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Alpha-1 antitrypsin

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404: 381: 2488: 2465: 278: 4648: 303: 1795: 4528: 4693: 4678: 4663: 2577:(FDA) has approved the use of four alpha-1 antitrypsin products derived from a human plasma: Prolastin, Zemaira, Glassia, and Aralast. These products for intravenous augmentation A1AT therapy can cost up to $ 100,000 per year per patient. They are administered intravenously at a dose of 60 mg/kg once a week; higher doses do not provide additional benefit although they can be used in anticipation of an interruption of weekly administration, such as for a vacation. 662: 655: 4618: 4588: 4573: 4543: 4603: 410: 309: 4498: 4513: 4633: 4483: 31: 4558: 2581:(null, null), PiSZ). People are to be under optimal pharmacologic and non-pharmacologic treatment and show evidence of progressive lung disease (e.g. lower forced expiratory volume per second (FEV1) predicted, impaired walking capacity or increased number of exacerbations) as evaluated by a healthcare professional experienced in the treatment of alpha1-proteinase inhibitor deficiency. 6149: 3677: 1832:-antitrypsin (382 in the pre-processed form containing the 24 amino acid signal peptide), a residue essential for binding elastase; this is thought to be one of the primary mechanisms by which cigarette smoking (or second-hand smoke) can lead to emphysema. Because A1AT is expressed in the liver, certain mutations in the 2587:
Aerosolized-augmented A1AT therapy is under study. This involves inhaling purified human A1AT into the lungs and trapping the A1AT into the lower respiratory tract. However, inhaled A1AT may not reach the elastin fibers in the lung where elastase injury occurs. Further study is currently underway.
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Alpha1-proteinase inhibitor (Respreeza) was approved for medical use in the European Union in August 2015. It is indicated for maintenance treatment, to slow the progression of emphysema in adults with documented severe alpha1-proteinase inhibitor deficiency (e.g., genotypes PiZZ, PiZ (null), Pi
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A1AT not only binds to neutrophil elastase from inflammatory cells but also to elastase on the cell surface. In this latter role, elastase acts as a signaling molecule for cell movement, rather than as an enzyme. Besides liver cells, A1PI is also produced in bone marrow, lymphoid tissue, and the
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Besides limiting elastase activity to limit tissue degradation, A1PI also acts to induce locomotion of lymphocytes through tissue including immature T cells through the thymus where immature T cells mature to become immunocompetent T cells that are released into tissue to elevate immune
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Inactivation of A1AT by other enzymes during inflammation or infection can halt T cell migration precisely at the site of the pathological insult. This suggests that α1PI plays a key role in lymphocyte movement and immune surveillance, particularly in response to infection. A1AT is both
57: 1906:. This suggests that A1AT may play an anti-inflammatory or tissue-protecting role outside the lungs. These antibodies are associated with a more severe disease course, can be observed years before disease onset, and may predict the development of RA in 1969:, as it migrates toward the center of such an IEF gel. Other variants are less functional and are termed A-L and N-Z, dependent on whether they run proximal or distal to the M band. The presence of deviant bands on IEF can signify the presence of 3149:
Verheul MK, Yee A, Seaman A, Janssen GM, van Veelen PA, Drijfhout JW, et al. (June 2017). "Identification of carbamylated alpha 1 anti-trypsin (A1AT) as an antigenic target of anti-CarP antibodies in patients with rheumatoid arthritis".
4647: 1973:. Since the number of identified mutations has exceeded the number of letters in the alphabet, subscripts have been added to most recent discoveries in this area, as in the Pittsburgh mutation described above. 3256: 4527: 1819:
in which a deficiency of alpha-1 antitrypsin leads to a chronic uninhibited tissue breakdown. This causes the degradation especially of lung tissue and eventually leads to characteristic manifestations of
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with two different copies of the gene may have two different bands showing on electrofocusing, although heterozygote with one null mutant that abolishes expression of the gene will only show one band.
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Alkins SA, O'Malley P (March 2000). "Should health-care systems pay for replacement therapy in patients with alpha(1)-antitrypsin deficiency? A critical review and cost-effectiveness analysis".
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Owen MC, Brennan SO, Lewis JH, Carrell RW (September 1983). "Mutation of antitrypsin to antithrombin. alpha 1-antitrypsin Pittsburgh (358 Met leads to Arg), a fatal bleeding disorder".
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The most common side effects include dizziness, headache, dyspnoea (shortness of breath) and nausea. Allergic reactions have been observed during treatment, some of which were severe.
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Kolarich D, Weber A, Turecek PL, Schwarz HP, Altmann F (June 2006). "Comprehensive glyco-proteomic analysis of human alpha1-antitrypsin and its charge isoforms".
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Carrell RW, Jeppsson JO, Laurell CB, Brennan SO, Owen MC, Vaughan L, et al. (July 1982). "Structure and variation of human alpha 1-antitrypsin".
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Kalsheker N, Morley S, Morgan K (April 2002). "Gene regulation of the serine proteinase inhibitors alpha1-antitrypsin and alpha1-antichymotrypsin".
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to measure how much A1AT is present. Other detection methods include the use of enzyme-linked-immuno-sorbent-assays and radial immunodiffusion.
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Coakley RJ, Taggart C, O'Neill S, McElvaney NG (January 2001). "Alpha1-antitrypsin deficiency: biological answers to clinical questions".
3026:"Alpha1-antitrypsin deficiency. 2: genetic aspects of alpha(1)-antitrypsin deficiency: phenotypes and genetic modifiers of emphysema risk" 1731:. These enzymes are released locally in relatively low concentrations where they are immediately cleared by proteins such as A1AT. In the 4497: 4422: 3662: 403: 54: 6189: 2161: 4512: 1248: 5561: 4404: 1241: 4130:
Lomas DA, Lourbakos A, Cumming SA, Belorgey D (April 2002). "Hypersensitive mousetraps, alpha1-antitrypsin deficiency and dementia".
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carry different amounts of negatively charged sialic acids; this causes the heterogeneity observed on normal A1AT when analysed by
1481:–proteinase inhibitor (human) and under various trade names (including Aralast NP, Glassia, Prolastin, Prolastin-C, and Zemaira). 3278: 2857:"Current developments in mobilization of hematopoietic stem and progenitor cells and their interaction with niches in bone marrow" 2808:"Serine protease inhibitors serpina1 and serpina3 are down-regulated in bone marrow during hematopoietic progenitor mobilization" 1929:
of 0.9–2.3 g/L (in the US the reference range is expressed as mg/dL or micromoles), but the concentration can rise manyfold upon
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Elliott PR, Abrahams JP, Lomas DA (January 1998). "Wild-type alpha 1-antitrypsin is in the canonical inhibitory conformation".
2673: 2652: 2303: 2216: 2142: 4428: 3075:"Oxidation of either methionine 351 or methionine 358 in alpha 1-antitrypsin causes loss of anti-neutrophil elastase activity" 1884: 6179: 6046: 5716: 5513: 2102: 2055: 1970: 1809: 1803: 1410: 302: 277: 4656:: Active site distortion is sufficient for proteinase inhibit second crystal structure of covalent serpin-proteinase complex 4632: 2669: 6174: 5554: 2759:"Acute-phase protein α1-anti-trypsin: diverting injurious innate and adaptive immune responses from non-authentic threats" 2648: 1617:
are most at risk for carrying one of the most common mutant forms of A1AT, the Z mutation (Glu342Lys on M1A, rs28929474).
6139: 5244: 3612: 3575: 3538: 3501: 3464: 3427: 2574: 2376: 2010: 1709: 1458: 1347: 416: 315: 219: 4536:: A 2.1 ANGSTROM STRUCTURE OF AN UNCLEAVED ALPHA-1-ANTITRYPSIN SHOWS VARIABILITY OF THE REACTIVE CENTER AND OTHER LOOPS 5659: 4482: 2444: 1958:(IEF) in the pH range 4.5-5.5, where the protein migrates in a gel according to its isoelectric point or charge in a 409: 308: 1497:
The protein was initially named "antitrypsin" because of its ability to bind and irreversibly inactivate the enzyme
5841: 5052: 5011: 3569: 2620:, another serpin that is analogous for protecting the body from excessive effects of its own inflammatory proteases 3421: 6002: 5709: 5593: 5508: 4455: 3656: 3532: 1865: 1759: 1633:. However, one particular site shows a considerable amount of heterogeneity since tri- and even tetraantennary N- 1798:
Alpha-1 antitrypsin (white) with highlighted 'reactive centre loop' (blue) and A-sheet (light blue). (PDB: 1QLP)
5851: 5614: 5119: 4818: 4756: 3731:"Intravenous Alpha-1 Antitrypsin Therapy for Alpha-1 Antitrypsin Deficiency: The Current State of the Evidence" 3606: 3495: 3458: 2617: 994: 227: 3218: 5402: 6085: 5870: 5179: 3829:"Recombinant Alpha-1 Antitrypsin as Dry Powder for Pulmonary Administration: A Formulative Proof of Concept" 2172: 1529: 975: 971: 4557: 2483: 2904: 2405: 1736: 1936:
The level of A1AT in serum is most often determined by adding an antibody that binds to A1AT, then using
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Kalsheker N (April 1989). "Alpha 1-antitrypsin: structure, function and molecular biology of the gene".
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region can be further divided into two sub-regions, termed "1" and "2". Alpha-1 antitrypsin is the main
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A1AT is a single-chain glycoprotein consisting of 394 amino acids in the mature form and exhibits many
4348:"Alpha-1-antitrypsin deficiency: a new paradigm for hepatocellular carcinoma in genetic liver disease" 5462: 5278: 4448: 3999: 2757:
Guttman O, Baranovski BM, Schuster R, Kaner Z, Freixo-Lima GS, Bahar N, et al. (February 2015).
2134: 1955: 1891: 1813: 1650: 1418: 206: 6104: 5685: 5619: 5577: 5457: 5397: 5143: 5133: 5123: 4907: 4808: 4786: 4781: 4766: 4761: 4701:: THE S VARIANT OF HUMAN ALPHA1-ANTITRYPSIN, STRUCTURE AND IMPLICATIONS FOR FUNCTION AND METABOLISM 4686:: THE S VARIANT OF HUMAN ALPHA1-ANTITRYPSIN, STRUCTURE AND IMPLICATIONS FOR FUNCTION AND METABOLISM 4671:: THE S VARIANT OF HUMAN ALPHA1-ANTITRYPSIN, STRUCTURE AND IMPLICATIONS FOR FUNCTION AND METABOLISM 4434: 4190:"Liver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury" 3073:
Taggart C, Cervantes-Laurean D, Kim G, McElvaney NG, Wehr N, Moss J, et al. (September 2000).
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Bianchera A, Alomari E, Michielon A, Bazzoli G, Ronda N, Pighini G, et al. (December 2022).
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Miyamoto Y, Akaike T, Maeda H (March 2000). "S-nitrosylated human alpha(1)-protease inhibitor".
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alpha-1 antitrypsin is not yet available for use as a medication but is under development.
2385: 1629:. The three N-linked glycosylations sites are mainly equipped with so-called diantennary N- 6153: 6080: 5837: 5833: 5829: 5745: 5732: 5639: 5598: 5234: 5155: 5016: 4408: 2589: 2329: 1951: 1837: 1482: 471: 2023:. Some mutant forms fail to fold properly and are, thus, targeted for destruction in the 4003: 2487: 2464: 5350: 5307: 5224: 5129: 5115: 5095: 4838: 4413: 4329: 4304: 3896: 3879: 3855: 3828: 3804: 3779: 3755: 3730: 3050: 3025: 2881: 2856: 2832: 2807: 2783: 2758: 1903: 1658: 1576: 1557: 1470: 4257: 4232: 4214: 4189: 4077: 3961:
Crystal RG (December 1989). "The alpha 1-antitrypsin gene and its deficiency states".
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Mackiewicz A, Kushner I, Baumann H, eds. (1993). "Acute phase response: an overview".
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encoding the protein can cause misfolding and impaired secretion, which can lead to
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Biochimica et Biophysica Acta (BBA) - Protein Structure and Molecular Enzymology
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Acute phase proteins: molecular biology, biochemistry, and clinical applications
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National Center for Biotechnology Information, U.S. National Library of Medicine
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National Center for Biotechnology Information, U.S. National Library of Medicine
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patients. Consequently, carbamylated A1AT is currently being developed as an
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ENSG00000277377 GRCh38: Ensembl release 89: ENSG00000197249, ENSG00000277377
2128: 2073: 2062: 1944: 1914: 1821: 1735:, a further elevation is required to "limit" the damage caused by activated 1673: 1642: 1626: 1506: 1442: 890: 632: 510: 455: 442: 354: 341: 243: 210:, A1A, A1AT, AAT, PI, PI1, PRO2275, alpha1AT, serpin family A member 1, nNIF 4373: 4338: 4295: 4266: 4223: 4180: 4151: 4114: 4085: 4048: 3864: 3813: 3764: 3715: 3171: 3100: 3091: 3074: 3059: 3010: 2961: 2953: 2890: 2841: 2792: 2743: 2084:
Other rarer forms have been described; in all, there are over 80 variants.
1441:. However, defective A1AT may accumulate in the liver, potentially causing 4056: 4019: 3982: 3945: 3905: 3135: 1288: 1283: 6114: 6041: 6012: 5885: 5880: 5701: 5644: 5315: 5283: 5086: 4947: 4942: 4927: 4922: 4917: 4885: 4880: 4875: 4870: 4860: 4855: 4845: 4833: 4828: 4823: 4813: 2823: 2722:
Gettins PG (December 2002). "Serpin structure, mechanism, and function".
2340: 2156: 2020: 1873: 1775: 1740: 1728: 1700: 1677: 1669: 1561: 1532:. On electrophoresis, the protein component of the blood is separated by 1510: 1502: 1375: 1272: 1043: 1039: 1020: 3780:"Feasibility of Aerosolized Alpha-1 Antitrypsin as a Therapeutic Option" 2349: 5585: 5546: 5412: 5355: 5288: 4172: 4143: 3937: 2028: 1948: 1911: 1779: 1748: 1665: 1661: 1646: 1634: 1630: 1613:-antitrypsin have been described in various populations. North-Western 1572: 1498: 1414: 1379: 1332: 1006: 957: 4596:: NON-COVALENT COMPLEX BETWEEN ALPHA-1-PI-PITTSBURGH AND S195A TRYPSIN 4364: 4347: 3335:"Health product highlights 2021: Annexes of products approved in 2021" 2872: 2806:
Winkler IG, Hendy J, Coughlin P, Horvath A, Lévesque JP (April 2005).
2774: 1653:. Also, the fucosylated triantennary N-glycans were shown to have the 1513:
and elsewhere. In older biomedical literature it was sometimes called
176: 172: 168: 164: 160: 156: 152: 148: 144: 140: 136: 132: 128: 124: 120: 116: 112: 108: 104: 100: 96: 92: 88: 84: 80: 76: 6093: 5649: 5439: 5105: 4996: 4957: 4865: 4742: 4397: 4248: 4205: 4011: 2735: 2605: 2396: 2077: 2066: 1977: 1876:). One person with this mutation has been reported to have died of a 1824:. Evidence has shown that cigarette smoke can result in oxidation of 1724: 1696: 1654: 1614: 1391: 1336: 1256: 912: 5044: 2911:. U.S. National Library of Medicine, National Insittues of Health. 5503: 2432: 1793: 1783: 1434: 1406: 4581:: CRYSTAL STRUCTURE OF A1PI-PITTSBURGH IN THE NATIVE CONFORMATION 4551:: Interactions causing the kinetic trap in serpin protein folding 4276:
Clinica Chimica Acta; International Journal of Clinical Chemistry
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This article incorporates text from this source, which is in the
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nomenclature) is found to be covalently linked to a free single
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Lisowska-Myjak B (February 2005). "AAT as a diagnostic tool".
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endoplasmic reticulum-Golgi intermediate compartment membrane
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in these areas can lead to non-functional proteins that can
4611:: INTACT RECOMBINED ALPHA1-ANTITRYPSIN MUTANT PHE 51 TO LEU 2225: 1959: 1668:
particular protein-cell recognition properties. The single
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Brantly ML, Lascano JE, Shahmohammadi A (November 2018).
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endoplasmic reticulum to Golgi vesicle-mediated transport
2238: 2035:. The serum levels of some of the common genotypes are: 1943:
Different analytical methods are used to determine A1AT
1703:, it is considered the most prominent serpin; the terms 479: 3389:"Respreeza - Summary of Product Characteristics (SmPC)" 2909:
ClinVar Genomic variation as it relates to human health
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Alpha-1 antitrypsin concentrates are prepared from the
2559: 1517:(STI, dated terminology), because its capability as a 1485:
versions are also available but are currently used in
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online database for peptidases and their inhibitors:
2905:"NM_001127701.1(SERPINA1):c.1096G>A (p.Glu366Lys)" 644: 2080:
mutation at position 264 (288 in pre-processed form)
2069:
mutation at position 342 (366 in pre-processed form)
6059: 5995: 5919: 5797: 5766: 5759: 5744: 5678: 5607: 5584: 5476: 5448: 5420: 5411: 5368: 5324: 5306: 5259: 5232: 5223: 5188: 5153: 5103: 5094: 5082: 4984: 4956: 4749: 3363:"Regulatory Decision Summary for Pronextica Liquid" 2538: 2499: 2494: 2475: 2443: 2415: 2395: 2375: 2359: 2339: 2319: 2294: 2215: 2210: 2185: 2171: 2141: 2123: 2113: 2108: 1209: 1148: 1122: 1061: 4506:: CRYSTAL STRUCTURE OF CLEAVED ANTITRYPSIN POLYMER 3880:"Hereditary variants of serum alpha-1-antitrypsin" 2665: 2663: 2661: 2644: 2642: 2640: 1887:-positive globules within periportal hepatocytes. 1413:), neutrophil elastase can excessively break down 3646: 3644: 3642: 3640: 3638: 3636: 2855:Richler R, Forssmann W, Henschler R (June 2017). 2013:and "MM" is the banding pattern of that patient. 426: 325: 4521:: CRYSTAL STRUCTURE OF A SERPIN:PROTEASE COMPLEX 2935: 2933: 1954:is imprecise, the A1AT phenotype is analysed by 2328: 1560:). The non-albumin proteins are referred to as 1433:, in adults. Normally, A1AT is produced in the 3564: 3562: 2670:GRCm38: Ensembl release 89: ENSMUSG00000071177 5717: 5562: 5060: 4727: 4456: 3416: 3414: 2608:variants of A1AT leading to disease in 1965. 1409:contains inadequate or defective A1AT (as in 846:negative regulation of endopeptidase activity 8: 4346:Rudnick DA, Perlmutter DH (September 2005). 4095:The American Journal of the Medical Sciences 3527: 3525: 2160: 2092: 1339:superfamily. It is encoded in humans by the 724:serine-type endopeptidase inhibitor activity 2287:In general: ℞ (Prescription only) 1995:results, the IEF results are notated as in 5763: 5756: 5724: 5710: 5702: 5569: 5555: 5547: 5417: 5229: 5100: 5091: 5067: 5053: 5045: 4734: 4720: 4712: 4463: 4449: 4441: 3601: 3599: 3490: 3488: 3453: 3451: 2486: 2463: 2100: 1521:was a salient feature of its early study. 1145: 1058: 886: 750:COPII-coated ER to Golgi transport vesicle 686: 467: 366: 263: 65: 6037:Tetragalacturonic acid hydroxymethylester 5535:disorders of globin and globulin proteins 5035:disorders of globin and globulin proteins 4363: 4328: 4256: 4213: 3895: 3854: 3844: 3803: 3754: 3090: 3049: 3000: 2880: 2831: 2782: 2384: 821:negative regulation of peptidase activity 3878:Axelsson U, Laurell CB (November 1965). 800:collagen-containing extracellular matrix 790:intracellular membrane-bounded organelle 6144: 4478: 2636: 2459: 2348: 2308: 2051:PiSZ: 40% of normal serum level of A1AT 2048:PiMZ: 60% of normal serum level of A1AT 2045:PiSS: 60% of normal serum level of A1AT 2042:PiMS: 80% of normal serum level of A1AT 861:post-translational protein modification 16:Mammalian protein found in Homo sapiens 4231:Lomas DA, Mahadeva R (December 2002). 3735:Chronic Obstructive Pulmonary Diseases 3547:from the original on 28 September 2020 3313:Therapeutic Goods Administration (TGA) 3283:Therapeutic Goods Administration (TGA) 3253:Therapeutic Goods Administration (TGA) 2925:Interpretation: Pathogenic risk factor 2763:Clinical & Experimental Immunology 2604:first investigated the possibility of 2477: 2091: 1509:, is a digestive enzyme active in the 20: 4641:: CLEAVED ALPHA-1-ANTITRYPSIN POLYMER 4237:The Journal of Clinical Investigation 4194:The Journal of Clinical Investigation 3219:"Alpha-1-Proteinase Inhibitor, Human" 3024:DeMeo DL, Silverman EK (March 2004). 2983:Lapidot T, Petit I (September 2012). 2425: 2404: 2133: 1431:chronic obstructive pulmonary disease 431: 392: 387: 330: 289: 284: 7: 4802:Protein Z-related protease inhibitor 2915:from the original on 13 October 2022 2812:The Journal of Experimental Medicine 2431: 2198: 2027:, whereas others have a tendency to 1528:refers to the protein's behavior on 3665:from the original on 3 October 2020 3584:from the original on 3 October 2020 3436:from the original on 10 August 2020 3399:from the original on 3 October 2020 3229:from the original on 3 October 2020 3116:The New England Journal of Medicine 3079:The Journal of Biological Chemistry 1463:an exogenous one used as medication 3884:American Journal of Human Genetics 3473:from the original on 4 August 2020 3289:from the original on 10 April 2023 3259:from the original on 10 April 2023 2019:levels in the blood depend on the 1883:A liver biopsy will show abundant 1808:Disorders of this protein include 1720:) are often used interchangeably. 1206: 1119: 1030: 1011: 985: 962: 936: 917: 649: 567: 505: 484: 14: 5912:(II, VII, IX, X, protein C and S) 4797:Plasminogen activator inhibitor-2 4792:Plasminogen activator inhibitor-1 3621:from the original on 1 April 2020 3510:from the original on 1 April 2020 2119:Aralast, Zemaira, Glassia, others 1672:residue of A1AT in position 256 ( 6147: 4691: 4676: 4661: 4646: 4631: 4616: 4601: 4586: 4571: 4556: 4541: 4526: 4511: 4496: 4481: 4161:Biochemical Society Transactions 4132:Biochemical Society Transactions 4107:10.1097/00000441-200101000-00006 3675: 2517: 2511: 1645:amino acid nomenclature). These 1609:Over 100 different variants of α 1459:an endogenous protease inhibitor 660: 653: 415: 408: 402: 379: 314: 307: 301: 276: 29: 5955:thrombopoietin receptor agonist 5910:Prothrombin complex concentrate 4491:: UNCLEAVED ALPHA-1-ANTITRYPSIN 4188:Perlmutter DH (December 2002). 3367:Drug and Health Products Portal 1786:(infantile hepatic cirrhosis). 6047:Hemostatic Powder Spray TC-325 5514:Extracellular matrix protein 1 2529: 2523: 2505: 2056:alpha-1 antitrypsin deficiency 1971:alpha-1 antitrypsin deficiency 1810:alpha-1 antitrypsin deficiency 1804:Alpha-1 antitrypsin deficiency 1602:is located on the long arm of 1411:alpha-1 antitrypsin deficiency 1374:) because it inhibits various 671:More reference expression data 633:More reference expression data 1: 4078:10.1016/S0167-4838(99)00264-2 3846:10.3390/pharmaceutics14122754 3796:10.15326/jcopdf.7.3.2019.0179 3747:10.15326/jcopdf.6.1.2017.0185 3002:10.1016/s0301-472x(02)00883-4 1894:(RA) have been found to make 400: 299: 6170:Genes on human chromosome 14 5245:Sex hormone-binding globulin 4321:10.7861/clinmedicine.5-3-249 4037:Journal of Molecular Biology 3975:10.1016/0168-9525(89)90200-X 3613:Food and Drug Administration 3576:Food and Drug Administration 3539:Food and Drug Administration 3502:Food and Drug Administration 3465:Food and Drug Administration 3428:Food and Drug Administration 3189:. CRC Press. pp. 3–19. 2626:is a related alpha 1 protein 2575:Food and Drug Administration 2311:Alpha-1-proteinase inhibitor 2162:Alpha 1 proteinase inhibitor 1762:, A1AT has a characteristic 1584:alpha-1 proteinase inhibitor 795:ficolin-1-rich granule lumen 765:platelet alpha granule lumen 704:peptidase inhibitor activity 5660:Haptoglobin-related protein 3128:10.1056/NEJM198309223091203 745:endoplasmic reticulum lumen 6206: 6190:Serine protease inhibitors 5842:Valoctocogene roxaparvovec 5012:Thyroxine-binding globulin 4433:gene details page in the 4288:10.1016/j.cccn.2004.03.012 3164:10.1016/j.jaut.2017.02.008 2495:Chemical and physical data 1843:An extremely rare form of 1801: 1760:serine protease inhibitors 1664:, which could confer this 1249:Chr 12: 103.73 – 103.74 Mb 6003:Absorbable gelatin sponge 5526: 5509:Vitamin D-binding protein 5030: 4476: 3657:European Medicines Agency 2705:"Mouse PubMed Reference:" 2687:"Human PubMed Reference:" 2555: 2299: 2099: 1489:more than as medication. 1453:Paneth cells of the gut. 1417:, leading to the loss of 1287: 1282: 1278: 1271: 1255: 1236: 1213: 1172: 1141: 1126: 1085: 1054: 1037: 1033: 1018: 1014: 1005: 992: 988: 969: 965: 956: 943: 939: 924: 920: 911: 896: 889: 885: 869: 719:identical protein binding 689: 685: 668: 652: 643: 630: 579: 570: 517: 508: 478: 470: 466: 449: 436: 399: 378: 369: 365: 348: 335: 298: 275: 266: 262: 217: 214: 204: 197: 192: 73: 68: 51: 46: 41: 37: 28: 23: 5852:Fidanacogene elaparvovec 5615:Alpha 1-antichymotrypsin 5120:Alpha 1-antichymotrypsin 4757:Alpha 1-antichymotrypsin 2618:Alpha 1-antichymotrypsin 2573:of blood donors. The US 2031:, being retained in the 1976:As every person has two 1927:reference range in blood 1743:, which breaks down the 1723:Most serpins inactivate 1242:Chr 14: 94.38 – 94.39 Mb 856:neutrophil degranulation 6086:Aminomethylbenzoic acid 5403:7S seed storage protein 5180:Retinol binding protein 3778:Usmani OS (July 2020). 3708:10.1378/chest.117.3.875 3152:Journal of Autoimmunity 3042:10.1136/thx.2003.006502 1737:neutrophil granulocytes 1657:as part of a so-called 1637:can be attached to the 1544:, the second being the 1530:protein electrophoresis 1515:serum trypsin inhibitor 553:upper lobe of left lung 4958:Cross class inhibitory 4049:10.1006/jmbi.1997.1458 3784:Chronic Obstr Pulm Dis 3092:10.1074/jbc.M004850200 2954:10.1002/pmic.200500751 1965:Normal A1AT is termed 1868:), due to a mutation ( 1799: 1782:and accumulate in the 1473:and is sold under the 1469:form is purified from 1350:, it is also known as 826:platelet degranulation 6180:Enzymes used as drugs 5818:Damoctocog alfa pegol 5625:Alpha 2-macroglobulin 5206:alpha-2-Macroglobulin 4992:Heat shock protein 47 3661:. 17 September 2018. 3580:. 21 September 2017. 2861:Transfus Med Hemother 2054:PiZZ: 10-15% (severe 2033:endoplasmic reticulum 1814:autosomal co-dominant 1797: 1790:Clinical significance 1582:Another name used is 1505:. Trypsin, a type of 1356:–proteinase inhibitor 836:COPII vesicle coating 775:endoplasmic reticulum 755:extracellular exosome 433:12 E|12 52.98 cM 394:Chromosome 12 (mouse) 292:Chromosome 14 (human) 6175:Acute-phase proteins 5578:Acute-phase proteins 5463:Bovine serum albumin 5279:Beta-2 microglobulin 4566:: ALPHA1-ANTITRYPSIN 4427:genome location and 4407:1 April 2008 at the 2824:10.1084/jem.20042299 1956:isoelectric focusing 1902:form of A1AT in the 1892:rheumatoid arthritis 1733:acute phase reaction 1651:isoelectric focusing 1536:. There are several 1439:systemic circulation 841:acute-phase response 770:extracellular region 587:hepatobiliary system 256:SERPINA1 - orthologs 69:List of PDB id codes 42:Available structures 5620:Alpha 1-antitrypsin 5458:Human serum albumin 5398:11S globulin family 5144:Heparin cofactor II 5134:Alpha 2-antiplasmin 5124:Alpha 1-antitrypsin 4787:Protein C inhibitor 4782:Heparin cofactor II 4767:Alpha 2-antiplasmin 4762:Alpha 1-antitrypsin 4435:UCSC Genome Browser 4416:Alpha-1-antitrypsin 4004:1982Natur.298..329C 3395:. 10 October 2018. 2263:(Prescription only) 2234:(Prescription only) 2096: 2094:Alpha-1 antitrypsin 2072:PiS is caused by a 2061:PiZ is caused by a 2039:PiMM: 100% (normal) 2017:Alpha-1 antitrypsin 1822:pulmonary emphysema 1817:hereditary disorder 1764:secondary structure 1727:by binding to them 1475:nonproprietary name 1445:in both adults and 1400:neutrophil elastase 1301:Alpha-1 antitrypsin 785:extracellular space 619:islet of Langerhans 529:islet of Langerhans 521:right lobe of liver 6032:Oxidized cellulose 5822:Efanesoctocog alfa 5630:C-reactive protein 5484:C-reactive protein 5376:Beta-lactoglobulin 4173:10.1042/BST0300093 4144:10.1042/BST0300089 3963:Trends in Genetics 3938:10.1007/BF01115992 3926:Bioscience Reports 3369:. 14 November 2023 2011:protease inhibitor 1931:acute inflammation 1878:bleeding diathesis 1860:, functions as an 1800: 1710:protease inhibitor 1540:, the first being 1427:respiratory issues 1425:. This results in 1398:cells, especially 1348:protease inhibitor 995:ENSMUSG00000071177 809:Biological process 733:Cellular component 697:Molecular function 6135: 6134: 6105:Alfa1 antitrypsin 6076:Aminocaproic acid 6061:Antifibrinolytics 6055: 6054: 5991: 5990: 5826:Efmoroctocog alfa 5776:Phytomenadione (K 5699: 5698: 5544: 5543: 5522: 5521: 5493:Alpha-lactalbumin 5393:Alpha-lactalbumin 5364: 5363: 5336:Fetal fibronectin 5302: 5301: 5219: 5218: 5076:Globular proteins 5042: 5041: 4709: 4708: 4365:10.1002/hep.20815 4309:Clinical Medicine 4303:Lomas DA (2005). 2873:10.1159/000477262 2775:10.1111/cei.12476 2567: 2566: 2445:CompTox Dashboard 2283: 2271: 2258: 2241: 2229: 2154: 1745:connective tissue 1739:and their enzyme 1519:trypsin inhibitor 1471:human donor blood 1382:). As a type of 1335:belonging to the 1298: 1297: 1294: 1293: 1267: 1266: 1232: 1231: 1203: 1202: 1137: 1136: 1116: 1115: 1050: 1049: 1027: 1026: 1001: 1000: 982: 981: 952: 951: 933: 932: 881: 880: 851:blood coagulation 681: 680: 677: 676: 639: 638: 626: 625: 564: 563: 462: 461: 361: 360: 188: 187: 184: 183: 52:Ortholog search: 6197: 6152: 6151: 6150: 6143: 6008:Calcium alginate 5764: 5757: 5746:Antihemorrhagics 5733:Antihemorrhagics 5726: 5719: 5712: 5703: 5571: 5564: 5557: 5548: 5418: 5262: 5238: 5235:carrier proteins 5230: 5191: 5159: 5156:carrier proteins 5109: 5101: 5092: 5069: 5062: 5055: 5046: 4736: 4729: 4722: 4713: 4695: 4680: 4665: 4650: 4635: 4620: 4605: 4590: 4575: 4560: 4545: 4530: 4515: 4500: 4485: 4465: 4458: 4451: 4442: 4385: 4367: 4342: 4332: 4299: 4270: 4260: 4249:10.1172/JCI16782 4227: 4217: 4206:10.1172/JCI16787 4184: 4155: 4126: 4089: 4060: 4031: 4012:10.1038/298329a0 3998:(5872): 329–34. 3986: 3957: 3910: 3909: 3899: 3875: 3869: 3868: 3858: 3848: 3824: 3818: 3817: 3807: 3775: 3769: 3768: 3758: 3726: 3720: 3719: 3691: 3685: 3679: 3678: 3674: 3672: 3670: 3652:"Respreeza EPAR" 3648: 3631: 3630: 3628: 3626: 3617:. 22 July 2017. 3603: 3594: 3593: 3591: 3589: 3566: 3557: 3556: 3554: 3552: 3543:. 5 March 2018. 3529: 3520: 3519: 3517: 3515: 3506:. 22 July 2017. 3492: 3483: 3482: 3480: 3478: 3469:. 22 July 2017. 3455: 3446: 3445: 3443: 3441: 3432:. 22 July 2017. 3418: 3409: 3408: 3406: 3404: 3385: 3379: 3378: 3376: 3374: 3359: 3353: 3352: 3350: 3348: 3331: 3325: 3324: 3322: 3320: 3305: 3299: 3298: 3296: 3294: 3285:. 21 June 2022. 3275: 3269: 3268: 3266: 3264: 3255:. 21 June 2022. 3245: 3239: 3238: 3236: 3234: 3215: 3209: 3208: 3182: 3176: 3175: 3146: 3140: 3139: 3111: 3105: 3104: 3094: 3085:(35): 27258–65. 3070: 3064: 3063: 3053: 3021: 3015: 3014: 3004: 2980: 2974: 2973: 2937: 2928: 2927: 2922: 2920: 2901: 2895: 2894: 2884: 2852: 2846: 2845: 2835: 2803: 2797: 2796: 2786: 2754: 2748: 2747: 2736:10.1021/cr010170 2730:(12): 4751–804. 2724:Chemical Reviews 2719: 2713: 2712: 2701: 2695: 2694: 2683: 2677: 2667: 2656: 2646: 2563: 2562: 2550: 2548: 2531: 2525: 2519: 2513: 2507: 2490: 2479: 2468: 2467: 2453: 2451: 2435: 2429: 2408: 2388: 2352: 2332: 2281: 2278: 2269: 2266: 2256: 2253: 2249: 2240: 2237: 2227: 2224: 2202: 2164: 2153: 2150: 2137: 2104: 2097: 2095: 1755:responsiveness. 1682:disulfide bridge 1534:electric current 1487:medical research 1384:enzyme inhibitor 1280: 1279: 1251: 1244: 1227: 1207: 1198: 1146: 1142:RefSeq (protein) 1132: 1120: 1111: 1059: 1031: 1012: 986: 963: 937: 918: 887: 709:protease binding 687: 673: 664: 657: 650: 635: 575: 573:Top expressed in 568: 513: 511:Top expressed in 506: 485: 468: 458: 445: 434: 419: 412: 406: 395: 383: 367: 357: 344: 333: 318: 311: 305: 294: 280: 264: 258: 209: 202: 179: 66: 60: 39: 38: 33: 21: 6205: 6204: 6200: 6199: 6198: 6196: 6195: 6194: 6160: 6159: 6158: 6148: 6146: 6138: 6136: 6131: 6081:Tranexamic acid 6051: 5987: 5921: 5915: 5838:Turoctocog alfa 5834:Susoctocog alfa 5830:Moroctocog alfa 5800: 5793: 5788: 5779: 5748: 5740: 5730: 5700: 5695: 5674: 5603: 5580: 5575: 5545: 5540: 5539: 5518: 5472: 5444: 5407: 5369:Other globulins 5360: 5320: 5316:Immunoglobulins 5298: 5260: 5255: 5252: 5233: 5215: 5189: 5184: 5154: 5149: 5104: 5096:Alpha globulins 5078: 5073: 5043: 5038: 5026: 5017:Angiotensinogen 4980: 4952: 4745: 4740: 4710: 4705: 4702: 4696: 4687: 4681: 4672: 4666: 4657: 4651: 4642: 4636: 4627: 4621: 4612: 4606: 4597: 4591: 4582: 4576: 4567: 4561: 4552: 4546: 4537: 4531: 4522: 4516: 4507: 4501: 4492: 4486: 4472: 4469: 4409:Wayback Machine 4393: 4388: 4345: 4302: 4273: 4243:(11): 1585–90. 4230: 4200:(11): 1579–83. 4187: 4158: 4129: 4092: 4063: 4034: 3989: 3960: 3923: 3919: 3917:Further reading 3914: 3913: 3877: 3876: 3872: 3826: 3825: 3821: 3777: 3776: 3772: 3728: 3727: 3723: 3693: 3692: 3688: 3676: 3668: 3666: 3650: 3649: 3634: 3624: 3622: 3605: 3604: 3597: 3587: 3585: 3568: 3567: 3560: 3550: 3548: 3531: 3530: 3523: 3513: 3511: 3494: 3493: 3486: 3476: 3474: 3457: 3456: 3449: 3439: 3437: 3420: 3419: 3412: 3402: 3400: 3387: 3386: 3382: 3372: 3370: 3361: 3360: 3356: 3346: 3344: 3343:. 3 August 2022 3333: 3332: 3328: 3318: 3316: 3307: 3306: 3302: 3292: 3290: 3277: 3276: 3272: 3262: 3260: 3247: 3246: 3242: 3232: 3230: 3217: 3216: 3212: 3197: 3184: 3183: 3179: 3148: 3147: 3143: 3113: 3112: 3108: 3072: 3071: 3067: 3023: 3022: 3018: 2982: 2981: 2977: 2948:(11): 3369–80. 2939: 2938: 2931: 2918: 2916: 2903: 2902: 2898: 2854: 2853: 2849: 2805: 2804: 2800: 2756: 2755: 2751: 2721: 2720: 2716: 2703: 2702: 2698: 2685: 2684: 2680: 2668: 2659: 2647: 2638: 2633: 2614: 2598: 2558: 2556: 2546: 2544: 2534: 2528: 2522: 2516: 2510: 2471: 2447: 2439: 2411: 2391: 2371: 2355: 2335: 2315: 2312: 2307: 2306: 2290: 2247: 2206: 2174: 2167: 2090: 2007: 2000: 1952:electrophoresis 1923: 1859: 1855: 1848: 1838:liver cirrhosis 1831: 1806: 1792: 1718: 1690: 1623: 1612: 1596: 1589: 1558:immunoglobulins 1552:and the fourth 1495: 1480: 1437:and enters the 1368:-antiproteinase 1367: 1355: 1320: 1308: 1289:View/Edit Mouse 1284:View/Edit Human 1247: 1240: 1237:Location (UCSC) 1223: 1219: 1215: 1194: 1190: 1186: 1182: 1178: 1174: 1168: 1164: 1160: 1156: 1152: 1128: 1107: 1103: 1099: 1095: 1091: 1087: 1081: 1077: 1073: 1069: 1065: 1042: 976:ENSG00000277377 974: 972:ENSG00000197249 865: 804: 740:Golgi apparatus 728: 714:protein binding 669: 659: 658: 631: 622: 617: 613: 609: 605: 601: 597: 593: 589: 585: 583:proximal tubule 571: 560: 555: 551: 547: 543: 539: 535: 531: 527: 523: 509: 453: 440: 432: 422: 421: 420: 413: 393: 370:Gene location ( 352: 339: 331: 321: 320: 319: 312: 290: 267:Gene location ( 218: 205: 198: 75: 53: 17: 12: 11: 5: 6203: 6201: 6193: 6192: 6187: 6182: 6177: 6172: 6162: 6161: 6157: 6156: 6133: 6132: 6130: 6129: 6128: 6127: 6119: 6118: 6117: 6112: 6107: 6102: 6090: 6089: 6088: 6083: 6078: 6065: 6063: 6057: 6056: 6053: 6052: 6050: 6049: 6044: 6039: 6034: 6029: 6024: 6015: 6010: 6005: 5999: 5997: 5993: 5992: 5989: 5988: 5986: 5985: 5984: 5983: 5978: 5973: 5968: 5963: 5951: 5946: 5941: 5936: 5931: 5925: 5923: 5917: 5916: 5914: 5913: 5903: 5902: 5893: 5888: 5883: 5874: 5873: 5860: 5859: 5845: 5811: 5805: 5803: 5795: 5794: 5792: 5791: 5786: 5782: 5777: 5772: 5770: 5761: 5754: 5742: 5741: 5731: 5729: 5728: 5721: 5714: 5706: 5697: 5696: 5694: 5693: 5688: 5682: 5680: 5676: 5675: 5673: 5672: 5667: 5662: 5657: 5652: 5647: 5642: 5637: 5632: 5627: 5622: 5617: 5611: 5609: 5608:Other positive 5605: 5604: 5602: 5601: 5596: 5590: 5588: 5582: 5581: 5576: 5574: 5573: 5566: 5559: 5551: 5542: 5541: 5538: 5537: 5528: 5527: 5524: 5523: 5520: 5519: 5517: 5516: 5511: 5506: 5501: 5496: 5486: 5480: 5478: 5474: 5473: 5471: 5470: 5465: 5460: 5454: 5452: 5446: 5445: 5443: 5442: 5437: 5432: 5426: 5424: 5415: 5409: 5408: 5406: 5405: 5400: 5395: 5390: 5385: 5384: 5383: 5372: 5370: 5366: 5365: 5362: 5361: 5359: 5358: 5353: 5351:Transcobalamin 5348: 5339: 5328: 5326: 5322: 5321: 5319: 5318: 5312: 5310: 5308:Gamma globulin 5304: 5303: 5300: 5299: 5297: 5296: 5291: 5286: 5281: 5276: 5271: 5265: 5263: 5257: 5256: 5254: 5253: 5247: 5241: 5239: 5227: 5225:Beta globulins 5221: 5220: 5217: 5216: 5214: 5213: 5202: 5194: 5192: 5186: 5185: 5183: 5182: 5177: 5170: 5162: 5160: 5151: 5150: 5148: 5147: 5137: 5127: 5112: 5110: 5098: 5089: 5080: 5079: 5074: 5072: 5071: 5064: 5057: 5049: 5040: 5039: 5031: 5028: 5027: 5025: 5024: 5019: 5014: 5009: 5004: 4999: 4994: 4988: 4986: 4982: 4981: 4979: 4978: 4973: 4968: 4962: 4960: 4954: 4953: 4951: 4950: 4945: 4940: 4935: 4930: 4925: 4920: 4915: 4910: 4905: 4899: 4898: 4893: 4888: 4883: 4878: 4873: 4868: 4863: 4858: 4853: 4848: 4842: 4841: 4836: 4831: 4826: 4821: 4816: 4811: 4805: 4804: 4799: 4794: 4789: 4784: 4779: 4774: 4769: 4764: 4759: 4753: 4751: 4747: 4746: 4741: 4739: 4738: 4731: 4724: 4716: 4707: 4706: 4704: 4703: 4697: 4690: 4688: 4682: 4675: 4673: 4667: 4660: 4658: 4652: 4645: 4643: 4637: 4630: 4628: 4622: 4615: 4613: 4607: 4600: 4598: 4592: 4585: 4583: 4577: 4570: 4568: 4562: 4555: 4553: 4547: 4540: 4538: 4532: 4525: 4523: 4517: 4510: 4508: 4502: 4495: 4493: 4487: 4480: 4477: 4474: 4473: 4470: 4468: 4467: 4460: 4453: 4445: 4439: 4438: 4419: 4411: 4392: 4391:External links 4389: 4387: 4386: 4343: 4300: 4271: 4228: 4185: 4156: 4127: 4090: 4061: 4032: 3987: 3958: 3920: 3918: 3915: 3912: 3911: 3870: 3819: 3770: 3741:(1): 100–114. 3721: 3686: 3632: 3595: 3558: 3521: 3484: 3447: 3410: 3380: 3354: 3326: 3315:. 21 June 2022 3300: 3270: 3240: 3225:. 4 May 2020. 3210: 3195: 3177: 3141: 3106: 3065: 3016: 2995:(9): 973–981. 2975: 2929: 2896: 2867:(3): 151–164. 2847: 2818:(7): 1077–88. 2798: 2769:(2): 161–172. 2749: 2714: 2696: 2678: 2657: 2635: 2634: 2632: 2629: 2628: 2627: 2621: 2613: 2610: 2597: 2594: 2565: 2564: 2553: 2552: 2542: 2536: 2535: 2532: 2526: 2520: 2514: 2508: 2503: 2497: 2496: 2492: 2491: 2481: 2473: 2472: 2470: 2469: 2456: 2454: 2441: 2440: 2438: 2437: 2421: 2419: 2413: 2412: 2410: 2409: 2401: 2399: 2393: 2392: 2390: 2389: 2381: 2379: 2373: 2372: 2370: 2369: 2365: 2363: 2357: 2356: 2354: 2353: 2345: 2343: 2337: 2336: 2334: 2333: 2325: 2323: 2317: 2316: 2314: 2313: 2310: 2302: 2301: 2300: 2297: 2296: 2292: 2291: 2289: 2288: 2285: 2276: 2264: 2251: 2235: 2221: 2219: 2213: 2212: 2208: 2207: 2205: 2204: 2191: 2189: 2183: 2182: 2177: 2175:administration 2169: 2168: 2166: 2165: 2147: 2145: 2139: 2138: 2131: 2121: 2120: 2117: 2111: 2110: 2106: 2105: 2089: 2086: 2082: 2081: 2070: 2059: 2052: 2049: 2046: 2043: 2040: 2005: 1998: 1922: 1919: 1904:synovial fluid 1896:autoantibodies 1890:Patients with 1857: 1853: 1846: 1829: 1802:Main article: 1791: 1788: 1716: 1705:α1-antitrypsin 1689: 1686: 1659:Sialyl Lewis x 1622: 1619: 1610: 1595: 1592: 1587: 1577:alpha-globulin 1494: 1491: 1478: 1467:pharmaceutical 1386:, it protects 1365: 1353: 1318: 1306: 1296: 1295: 1292: 1291: 1286: 1276: 1275: 1269: 1268: 1265: 1264: 1262: 1260: 1253: 1252: 1245: 1238: 1234: 1233: 1230: 1229: 1211: 1210: 1204: 1201: 1200: 1170: 1169: 1143: 1139: 1138: 1135: 1134: 1124: 1123: 1117: 1114: 1113: 1083: 1082: 1056: 1052: 1051: 1048: 1047: 1035: 1034: 1028: 1025: 1024: 1016: 1015: 1009: 1003: 1002: 999: 998: 990: 989: 983: 980: 979: 967: 966: 960: 954: 953: 950: 949: 941: 940: 934: 931: 930: 922: 921: 915: 909: 908: 903: 898: 894: 893: 883: 882: 879: 878: 867: 866: 864: 863: 858: 853: 848: 843: 838: 833: 828: 823: 818: 812: 810: 806: 805: 803: 802: 797: 792: 787: 782: 780:Golgi membrane 777: 772: 767: 762: 757: 752: 747: 742: 736: 734: 730: 729: 727: 726: 721: 716: 711: 706: 700: 698: 694: 693: 683: 682: 679: 678: 675: 674: 666: 665: 647: 641: 640: 637: 636: 628: 627: 624: 623: 621: 620: 616: 612: 608: 604: 600: 596: 592: 588: 584: 580: 577: 576: 565: 562: 561: 559: 558: 554: 550: 546: 542: 538: 534: 530: 526: 522: 518: 515: 514: 502: 501: 493: 482: 476: 475: 472:RNA expression 464: 463: 460: 459: 451: 447: 446: 438: 435: 430: 424: 423: 414: 407: 401: 397: 396: 391: 385: 384: 376: 375: 363: 362: 359: 358: 350: 346: 345: 337: 334: 329: 323: 322: 313: 306: 300: 296: 295: 288: 282: 281: 273: 272: 260: 259: 216: 212: 211: 203: 195: 194: 190: 189: 186: 185: 182: 181: 71: 70: 62: 61: 50: 44: 43: 35: 34: 26: 25: 15: 13: 10: 9: 6: 4: 3: 2: 6202: 6191: 6188: 6186: 6183: 6181: 6178: 6176: 6173: 6171: 6168: 6167: 6165: 6155: 6145: 6141: 6126: 6123: 6122: 6120: 6116: 6113: 6111: 6108: 6106: 6103: 6101: 6098: 6097: 6096: 6095: 6091: 6087: 6084: 6082: 6079: 6077: 6074: 6073: 6072: 6071: 6067: 6066: 6064: 6062: 6058: 6048: 6045: 6043: 6040: 6038: 6035: 6033: 6030: 6028: 6025: 6023: 6019: 6016: 6014: 6011: 6009: 6006: 6004: 6001: 6000: 5998: 5994: 5982: 5981:Rafutrombopag 5979: 5977: 5976:Lusutrombopag 5974: 5972: 5969: 5967: 5964: 5962: 5959: 5958: 5957: 5956: 5952: 5950: 5947: 5945: 5942: 5940: 5937: 5935: 5934:Carbazochrome 5932: 5930: 5927: 5926: 5924: 5918: 5911: 5908: 5907:combinations: 5905: 5904: 5901: 5897: 5894: 5892: 5889: 5887: 5884: 5882: 5879: 5876: 5875: 5872: 5868: 5865: 5862: 5861: 5857: 5853: 5849: 5846: 5843: 5839: 5835: 5831: 5827: 5823: 5819: 5815: 5812: 5810: 5807: 5806: 5804: 5802: 5796: 5790: 5783: 5781: 5774: 5773: 5771: 5769: 5765: 5762: 5758: 5755: 5752: 5747: 5743: 5738: 5734: 5727: 5722: 5720: 5715: 5713: 5708: 5707: 5704: 5692: 5689: 5687: 5686:Serum albumin 5684: 5683: 5681: 5677: 5671: 5668: 5666: 5663: 5661: 5658: 5656: 5653: 5651: 5648: 5646: 5643: 5641: 5638: 5636: 5635:Ceruloplasmin 5633: 5631: 5628: 5626: 5623: 5621: 5618: 5616: 5613: 5612: 5610: 5606: 5600: 5597: 5595: 5592: 5591: 5589: 5587: 5583: 5579: 5572: 5567: 5565: 5560: 5558: 5553: 5552: 5549: 5536: 5533: 5530: 5529: 5525: 5515: 5512: 5510: 5507: 5505: 5502: 5500: 5497: 5494: 5490: 5487: 5485: 5482: 5481: 5479: 5475: 5469: 5466: 5464: 5461: 5459: 5456: 5455: 5453: 5451: 5450:Serum albumin 5447: 5441: 5438: 5436: 5433: 5431: 5428: 5427: 5425: 5423: 5419: 5416: 5414: 5410: 5404: 5401: 5399: 5396: 5394: 5391: 5389: 5388:Thyroglobulin 5386: 5382: 5379: 5378: 5377: 5374: 5373: 5371: 5367: 5357: 5354: 5352: 5349: 5347: 5346:Microglobulin 5343: 5342:Macroglobulin 5340: 5337: 5333: 5330: 5329: 5327: 5323: 5317: 5314: 5313: 5311: 5309: 5305: 5295: 5292: 5290: 5287: 5285: 5282: 5280: 5277: 5275: 5272: 5270: 5267: 5266: 5264: 5258: 5251: 5248: 5246: 5243: 5242: 5240: 5236: 5231: 5228: 5226: 5222: 5211: 5207: 5203: 5200: 5196: 5195: 5193: 5187: 5181: 5178: 5175: 5174:Ceruloplasmin 5171: 5168: 5164: 5163: 5161: 5157: 5152: 5145: 5141: 5138: 5135: 5131: 5128: 5125: 5121: 5117: 5114: 5113: 5111: 5107: 5102: 5099: 5097: 5093: 5090: 5088: 5085: 5081: 5077: 5070: 5065: 5063: 5058: 5056: 5051: 5050: 5047: 5037: 5036: 5029: 5023: 5020: 5018: 5015: 5013: 5010: 5008: 5005: 5003: 5000: 4998: 4995: 4993: 4990: 4989: 4987: 4985:noninhibitory 4983: 4977: 4974: 4972: 4969: 4967: 4964: 4963: 4961: 4959: 4955: 4949: 4946: 4944: 4941: 4939: 4936: 4934: 4931: 4929: 4926: 4924: 4921: 4919: 4916: 4914: 4911: 4909: 4906: 4904: 4901: 4900: 4897: 4894: 4892: 4889: 4887: 4884: 4882: 4879: 4877: 4874: 4872: 4869: 4867: 4864: 4862: 4859: 4857: 4854: 4852: 4849: 4847: 4844: 4843: 4840: 4837: 4835: 4832: 4830: 4827: 4825: 4822: 4820: 4817: 4815: 4812: 4810: 4807: 4806: 4803: 4800: 4798: 4795: 4793: 4790: 4788: 4785: 4783: 4780: 4778: 4775: 4773: 4770: 4768: 4765: 4763: 4760: 4758: 4755: 4754: 4752: 4748: 4744: 4737: 4732: 4730: 4725: 4723: 4718: 4717: 4714: 4700: 4694: 4689: 4685: 4679: 4674: 4670: 4664: 4659: 4655: 4649: 4644: 4640: 4634: 4629: 4625: 4619: 4614: 4610: 4604: 4599: 4595: 4589: 4584: 4580: 4574: 4569: 4565: 4559: 4554: 4550: 4544: 4539: 4535: 4529: 4524: 4520: 4514: 4509: 4505: 4499: 4494: 4490: 4484: 4479: 4475: 4466: 4461: 4459: 4454: 4452: 4447: 4446: 4443: 4436: 4432: 4431: 4426: 4425: 4420: 4418: 4417: 4414:Proteopedia: 4412: 4410: 4406: 4403: 4399: 4395: 4394: 4390: 4383: 4379: 4375: 4371: 4366: 4361: 4358:(3): 514–21. 4357: 4353: 4349: 4344: 4340: 4336: 4331: 4326: 4322: 4318: 4315:(3): 249–57. 4314: 4310: 4306: 4301: 4297: 4293: 4289: 4285: 4282:(1–2): 1–13. 4281: 4277: 4272: 4268: 4264: 4259: 4254: 4250: 4246: 4242: 4238: 4234: 4229: 4225: 4221: 4216: 4211: 4207: 4203: 4199: 4195: 4191: 4186: 4182: 4178: 4174: 4170: 4166: 4162: 4157: 4153: 4149: 4145: 4141: 4137: 4133: 4128: 4124: 4120: 4116: 4112: 4108: 4104: 4100: 4096: 4091: 4087: 4083: 4079: 4075: 4072:(1–2): 90–7. 4071: 4067: 4062: 4058: 4054: 4050: 4046: 4043:(3): 419–25. 4042: 4038: 4033: 4029: 4025: 4021: 4017: 4013: 4009: 4005: 4001: 3997: 3993: 3988: 3984: 3980: 3976: 3972: 3969:(12): 411–7. 3968: 3964: 3959: 3955: 3951: 3947: 3943: 3939: 3935: 3932:(2): 129–38. 3931: 3927: 3922: 3921: 3916: 3907: 3903: 3898: 3893: 3890:(6): 466–72. 3889: 3885: 3881: 3874: 3871: 3866: 3862: 3857: 3852: 3847: 3842: 3838: 3834: 3833:Pharmaceutics 3830: 3823: 3820: 3815: 3811: 3806: 3801: 3797: 3793: 3789: 3785: 3781: 3774: 3771: 3766: 3762: 3757: 3752: 3748: 3744: 3740: 3736: 3732: 3725: 3722: 3717: 3713: 3709: 3705: 3702:(3): 875–80. 3701: 3697: 3690: 3687: 3683: 3682:public domain 3664: 3660: 3658: 3653: 3647: 3645: 3643: 3641: 3639: 3637: 3633: 3620: 3616: 3614: 3608: 3602: 3600: 3596: 3583: 3579: 3577: 3571: 3570:"Prolastin-C" 3565: 3563: 3559: 3546: 3542: 3540: 3534: 3528: 3526: 3522: 3509: 3505: 3503: 3497: 3491: 3489: 3485: 3472: 3468: 3466: 3460: 3454: 3452: 3448: 3435: 3431: 3429: 3423: 3417: 3415: 3411: 3398: 3394: 3390: 3384: 3381: 3368: 3364: 3358: 3355: 3342: 3341: 3340:Health Canada 3336: 3330: 3327: 3314: 3310: 3304: 3301: 3288: 3284: 3280: 3274: 3271: 3258: 3254: 3250: 3244: 3241: 3228: 3224: 3220: 3214: 3211: 3206: 3202: 3198: 3196:1-000-14197-7 3192: 3188: 3181: 3178: 3173: 3169: 3165: 3161: 3157: 3153: 3145: 3142: 3137: 3133: 3129: 3125: 3122:(12): 694–8. 3121: 3117: 3110: 3107: 3102: 3098: 3093: 3088: 3084: 3080: 3076: 3069: 3066: 3061: 3057: 3052: 3047: 3043: 3039: 3036:(3): 259–64. 3035: 3031: 3027: 3020: 3017: 3012: 3008: 3003: 2998: 2994: 2990: 2986: 2979: 2976: 2971: 2967: 2963: 2959: 2955: 2951: 2947: 2943: 2936: 2934: 2930: 2926: 2914: 2910: 2906: 2900: 2897: 2892: 2888: 2883: 2878: 2874: 2870: 2866: 2862: 2858: 2851: 2848: 2843: 2839: 2834: 2829: 2825: 2821: 2817: 2813: 2809: 2802: 2799: 2794: 2790: 2785: 2780: 2776: 2772: 2768: 2764: 2760: 2753: 2750: 2745: 2741: 2737: 2733: 2729: 2725: 2718: 2715: 2710: 2706: 2700: 2697: 2692: 2688: 2682: 2679: 2675: 2671: 2666: 2664: 2662: 2658: 2654: 2650: 2645: 2643: 2641: 2637: 2630: 2625: 2622: 2619: 2616: 2615: 2611: 2609: 2607: 2603: 2600:Axelsson and 2595: 2593: 2591: 2585: 2582: 2578: 2576: 2572: 2561: 2554: 2543: 2541: 2537: 2504: 2502: 2498: 2493: 2489: 2485: 2482: 2480: 2478:ECHA InfoCard 2474: 2466: 2462: 2461:DTXSID2040810 2458: 2457: 2455: 2446: 2442: 2434: 2433:RCSB PDB 2428: 2423: 2422: 2420: 2418: 2414: 2407: 2406:ChEMBL4297879 2403: 2402: 2400: 2398: 2394: 2387: 2383: 2382: 2380: 2378: 2374: 2367: 2366: 2364: 2362: 2358: 2351: 2347: 2346: 2344: 2342: 2338: 2331: 2327: 2326: 2324: 2322: 2318: 2309: 2305: 2298: 2293: 2286: 2284: Rx-only 2277: 2275: 2265: 2262: 2252: 2245: 2236: 2233: 2223: 2222: 2220: 2218: 2214: 2209: 2201: 2196: 2193: 2192: 2190: 2188: 2184: 2181: 2178: 2176: 2170: 2163: 2158: 2149: 2148: 2146: 2144: 2140: 2136: 2132: 2130: 2126: 2122: 2118: 2116: 2112: 2109:Clinical data 2107: 2103: 2098: 2087: 2085: 2079: 2075: 2071: 2068: 2064: 2060: 2057: 2053: 2050: 2047: 2044: 2041: 2038: 2037: 2036: 2034: 2030: 2026: 2022: 2018: 2014: 2012: 2008: 2001: 1994: 1989: 1987: 1983: 1979: 1974: 1972: 1968: 1963: 1961: 1957: 1953: 1950: 1946: 1941: 1939: 1934: 1932: 1928: 1920: 1918: 1916: 1913: 1909: 1905: 1901: 1897: 1893: 1888: 1886: 1881: 1879: 1875: 1871: 1867: 1863: 1856: 1849: 1841: 1839: 1835: 1827: 1823: 1818: 1815: 1811: 1805: 1796: 1789: 1787: 1785: 1781: 1777: 1773: 1772:alpha helices 1769: 1765: 1761: 1756: 1752: 1750: 1746: 1742: 1738: 1734: 1730: 1726: 1721: 1719: 1712: 1711: 1706: 1702: 1698: 1695: 1692:A1AT is a 52- 1687: 1685: 1683: 1679: 1675: 1671: 1667: 1663: 1660: 1656: 1652: 1648: 1644: 1640: 1636: 1632: 1628: 1620: 1618: 1616: 1607: 1605: 1604:chromosome 14 1601: 1593: 1591: 1585: 1580: 1578: 1574: 1570: 1565: 1563: 1559: 1555: 1551: 1547: 1543: 1539: 1535: 1531: 1527: 1522: 1520: 1516: 1512: 1508: 1504: 1500: 1492: 1490: 1488: 1484: 1476: 1472: 1468: 1464: 1460: 1454: 1450: 1448: 1444: 1440: 1436: 1432: 1428: 1424: 1420: 1416: 1412: 1408: 1403: 1401: 1397: 1393: 1389: 1385: 1381: 1377: 1373: 1369: 1361: 1357: 1349: 1345: 1342: 1338: 1334: 1330: 1326: 1322: 1314: 1310: 1302: 1290: 1285: 1281: 1277: 1274: 1270: 1263: 1261: 1258: 1254: 1250: 1246: 1243: 1239: 1235: 1228: 1226: 1222: 1218: 1212: 1208: 1205: 1199: 1197: 1193: 1189: 1185: 1181: 1177: 1171: 1167: 1163: 1159: 1155: 1151: 1147: 1144: 1140: 1133: 1131: 1125: 1121: 1118: 1112: 1110: 1106: 1102: 1098: 1094: 1090: 1084: 1080: 1076: 1072: 1068: 1064: 1060: 1057: 1055:RefSeq (mRNA) 1053: 1046: 1045: 1041: 1036: 1032: 1029: 1023: 1022: 1017: 1013: 1010: 1008: 1004: 997: 996: 991: 987: 984: 978: 977: 973: 968: 964: 961: 959: 955: 948: 947: 942: 938: 935: 929: 928: 923: 919: 916: 914: 910: 907: 904: 902: 899: 895: 892: 888: 884: 877: 873: 868: 862: 859: 857: 854: 852: 849: 847: 844: 842: 839: 837: 834: 832: 829: 827: 824: 822: 819: 817: 814: 813: 811: 808: 807: 801: 798: 796: 793: 791: 788: 786: 783: 781: 778: 776: 773: 771: 768: 766: 763: 761: 758: 756: 753: 751: 748: 746: 743: 741: 738: 737: 735: 732: 731: 725: 722: 720: 717: 715: 712: 710: 707: 705: 702: 701: 699: 696: 695: 692: 691:Gene ontology 688: 684: 672: 667: 663: 656: 651: 648: 646: 642: 634: 629: 618: 614: 610: 607:lobe of liver 606: 602: 598: 594: 590: 586: 582: 581: 578: 574: 569: 566: 556: 552: 548: 544: 540: 536: 532: 528: 524: 520: 519: 516: 512: 507: 504: 503: 500: 498: 494: 492: 491: 487: 486: 483: 481: 477: 473: 469: 465: 457: 452: 448: 444: 439: 429: 425: 418: 411: 405: 398: 390: 386: 382: 377: 373: 368: 364: 356: 351: 347: 343: 338: 328: 324: 317: 310: 304: 297: 293: 287: 283: 279: 274: 270: 265: 261: 257: 253: 249: 245: 241: 237: 233: 229: 225: 221: 213: 208: 201: 196: 191: 180: 178: 174: 170: 166: 162: 158: 154: 150: 146: 142: 138: 134: 130: 126: 122: 118: 114: 110: 106: 102: 98: 94: 90: 86: 82: 78: 72: 67: 64: 63: 59: 56: 49: 45: 40: 36: 32: 27: 22: 19: 6185:Orphan drugs 6110:C1-inhibitor 6092: 6068: 5966:Avatrombopag 5953: 5949:Fostamatinib 5906: 5900:Catridecacog 5891:I/Fibrinogen 5877: 5871:Eptacog alfa 5863: 5856:Nonacog alfa 5808: 5785:Menadione (K 5531: 5140:Antithrombin 5032: 4777:C1-inhibitor 4772:Antithrombin 4698: 4683: 4668: 4653: 4638: 4623: 4608: 4593: 4578: 4563: 4548: 4533: 4518: 4503: 4488: 4429: 4423: 4415: 4355: 4351: 4312: 4308: 4279: 4275: 4240: 4236: 4197: 4193: 4164: 4160: 4138:(2): 89–92. 4135: 4131: 4101:(1): 33–41. 4098: 4094: 4069: 4065: 4040: 4036: 3995: 3991: 3966: 3962: 3929: 3925: 3887: 3883: 3873: 3839:(12): 2754. 3836: 3832: 3822: 3790:(3): 272–9. 3787: 3783: 3773: 3738: 3734: 3724: 3699: 3695: 3689: 3667:. Retrieved 3655: 3623:. Retrieved 3610: 3586:. Retrieved 3573: 3549:. Retrieved 3536: 3512:. Retrieved 3499: 3475:. Retrieved 3462: 3438:. Retrieved 3425: 3422:"Aralast NP" 3401:. Retrieved 3392: 3383: 3371:. Retrieved 3366: 3357: 3345:. Retrieved 3338: 3329: 3317:. Retrieved 3312: 3303: 3291:. Retrieved 3282: 3273: 3261:. Retrieved 3252: 3243: 3231:. Retrieved 3222: 3213: 3186: 3180: 3155: 3151: 3144: 3119: 3115: 3109: 3082: 3078: 3068: 3033: 3029: 3019: 2992: 2988: 2978: 2945: 2941: 2924: 2917:. Retrieved 2908: 2899: 2864: 2860: 2850: 2815: 2811: 2801: 2766: 2762: 2752: 2727: 2723: 2717: 2708: 2699: 2690: 2681: 2599: 2586: 2583: 2579: 2571:blood plasma 2568: 2557:   2217:Legal status 2211:Legal status 2143:License data 2088:Medical uses 2083: 2016: 2015: 2003: 1996: 1990: 1986:heterozygote 1980:of the A1AT 1975: 1966: 1964: 1942: 1938:turbidimetry 1935: 1924: 1900:carbamylated 1889: 1882: 1862:antithrombin 1851: 1844: 1842: 1807: 1757: 1753: 1722: 1714: 1708: 1704: 1691: 1624: 1608: 1597: 1583: 1581: 1568: 1566: 1553: 1549: 1548:, the third 1545: 1537: 1525: 1523: 1514: 1496: 1493:Nomenclature 1455: 1451: 1404: 1396:inflammatory 1371: 1363: 1359: 1351: 1340: 1328: 1324: 1316: 1312: 1309:-antitrypsin 1304: 1300: 1299: 1221:NP_001239498 1214: 1196:NP_001121179 1192:NP_001121178 1188:NP_001121177 1184:NP_001121176 1180:NP_001121175 1176:NP_001121174 1173: 1166:NP_001121173 1162:NP_001121172 1158:NP_001002236 1154:NP_001002235 1127: 1109:NM_001127706 1105:NM_001127705 1101:NM_001127704 1097:NM_001127703 1093:NM_001127702 1089:NM_001127701 1086: 1079:NM_001127700 1075:NM_001002236 1071:NM_001002235 1063:NM_001127707 1038: 1019: 993: 970: 944: 925: 905: 900: 599:human kidney 595:right kidney 549:human kidney 495: 488: 454:103,739,851 441:103,729,853 215:External IDs 74: 18: 6125:Ulinastatin 6070:amino acids 6027:Fibrin glue 6018:Epinephrine 5971:Eltrombopag 5961:Romiplostim 5886:II/Thrombin 5799:Coagulation 5751:coagulation 5691:Transferrin 5670:Orosomucoid 5655:Haptoglobin 5499:Parvalbumin 5489:Lactalbumin 5381:Lactoferrin 5332:Fibronectin 5289:Plasminogen 5269:Angiostatin 5250:Transferrin 5210:Haptoglobin 5199:Orosomucoid 5167:Transcortin 5007:Transcortin 4471:PDB gallery 4167:(2): 93–8. 3533:"Prolastin" 2989:Exp Hematol 2624:Orosomucoid 2590:Recombinant 2551: g·mol 2484:100.029.919 2295:Identifiers 2180:Intravenous 2115:Trade names 2009:stands for 1925:A1AT has a 1898:toward the 1864:(a related 1768:beta sheets 1606:(14q32.1). 1483:Recombinant 541:gallbladder 537:granulocyte 353:94,390,693 340:94,376,747 193:Identifiers 6164:Categories 6022:adrenalone 5944:Etamsylate 5939:Concizumab 5929:Batroxobin 5864:extrinsic: 5809:intrinsic: 5468:Prealbumin 5430:Conalbumin 4750:inhibitory 4352:Hepatology 3205:1164833220 2942:Proteomics 2919:13 October 2676:, May 2017 2655:, May 2017 2631:References 2540:Molar mass 2417:PDB ligand 2386:F43I396OIS 2361:ChemSpider 2321:CAS Number 2304:IUPAC name 2250:Schedule D 2029:polymerise 2025:proteasome 2002:MM, where 1993:blood test 1962:gradient. 1908:arthralgia 1858:Pittsburgh 1826:methionine 1780:polymerise 1729:covalently 1639:Asparagine 1627:glycoforms 1579:1 region. 1503:covalently 1429:, such as 1419:elasticity 1378:(not just 816:hemostasis 499:(ortholog) 236:HomoloGene 6121:unsorted 6100:Aprotinin 5768:Vitamin K 5665:Hemopexin 5435:Ovalbumin 5422:Egg white 5294:Properdin 5274:Hemopexin 5204:alpha-2 ( 5197:alpha-1 ( 5172:alpha-2 ( 5165:alpha-1 ( 5087:globulins 5033:see also 5002:Ovalbumin 4896:SERPINB13 4891:SERPINB10 4839:SERPINA14 3607:"Zemaira" 3496:"Glassia" 3459:"Aralast" 3223:Drugs.com 3158:: 77–84. 2330:9041-92-3 2173:Routes of 2135:Monograph 2129:Drugs.com 2074:glutamate 2063:glutamate 1945:phenotype 1915:biomarker 1912:antigenic 1850:, termed 1776:Mutations 1758:Like all 1674:UniProtKB 1643:UniProtKB 1621:Structure 1615:Europeans 1562:globulins 1524:The term 1507:peptidase 1501:in vitro 1443:cirrhosis 1405:When the 1376:proteases 1225:NP_033269 1217:NP_033272 1150:NP_000286 1130:NM_009246 1067:NM_000295 891:Orthologs 244:GeneCards 6154:Medicine 6115:Camostat 6042:Thrombin 6013:Collagen 5922:systemic 5760:Systemic 5679:Negative 5645:Ferritin 5532:see also 5413:Albumins 5284:Factor H 4948:SERPINI2 4943:SERPINI1 4938:SERPINH1 4933:SERPING1 4928:SERPINF1 4923:SERPINE2 4918:SERPINE2 4913:SERPINE1 4908:SERPIND1 4903:SERPINC1 4886:SERPINB9 4881:SERPINB8 4876:SERPINB7 4871:SERPINB6 4866:SERPINB5 4861:SERPINB4 4856:SERPINB3 4851:SERPINB2 4846:SERPINB1 4834:SERPINA9 4829:SERPINA5 4824:SERPINA4 4819:SERPINA3 4814:SERPINA2 4809:SERPINA1 4430:SERPINA1 4424:SERPINA1 4405:Archived 4382:37875821 4374:16044402 4339:16011217 4296:15653097 4267:12464660 4224:12464659 4181:12023832 4152:12023831 4115:11202478 4086:10708851 4028:11904305 3954:34243822 3865:36559248 3814:32726075 3765:30775428 3716:10713018 3663:Archived 3619:Archived 3582:Archived 3545:Archived 3508:Archived 3471:Archived 3434:Archived 3397:Archived 3347:25 March 3319:31 March 3293:10 April 3287:Archived 3257:Archived 3227:Archived 3172:28291659 3101:10867014 3060:14985567 3011:12225788 2970:25498702 2962:16622833 2913:Archived 2891:28626366 2842:15795238 2793:25351931 2744:12475206 2672:– 2651:– 2612:See also 2560:(verify) 2341:DrugBank 2187:ATC code 2157:DailyMed 2021:genotype 1921:Analysis 1917:for RA. 1828:358 of α 1741:elastase 1701:medicine 1699:and, in 1688:Function 1678:cysteine 1670:cysteine 1594:Genetics 1538:clusters 1511:duodenum 1447:children 1341:SERPINA1 1273:Wikidata 870:Sources: 611:yolk sac 557:appendix 545:duodenum 533:monocyte 332:14q32.13 248:SERPINA1 207:SERPINA1 24:SERPINA1 6094:serpins 5878:common: 5801:factors 5586:Amyloid 5356:Edestin 5130:alpha-2 5116:alpha-1 5106:serpins 4743:Serpins 4402:I04.001 4330:4952210 4123:2458903 4057:9466920 4020:7045697 4000:Bibcode 3983:2696185 3946:2669992 3906:4158556 3897:1932630 3856:9784676 3805:7857706 3756:6373587 3373:1 April 3263:9 April 3136:6604220 3051:1746953 2882:5473067 2833:2213124 2784:4298394 2674:Ensembl 2653:Ensembl 2606:allelic 2602:Laurell 2596:History 2501:Formula 2350:DB00058 2246: / 2242:: 2203:) 2197: ( 2195:B02AB02 2159::  1949:protein 1749:elastin 1725:enzymes 1666:protein 1662:epitope 1647:glycans 1635:glycans 1631:glycans 1575:of the 1573:protein 1542:albumin 1526:alpha-1 1499:trypsin 1421:in the 1415:elastin 1392:enzymes 1388:tissues 1380:trypsin 1333:protein 1331:) is a 1007:UniProt 958:Ensembl 897:Species 876:QuickGO 603:Hindgut 474:pattern 200:Aliases 6140:Portal 5650:Fibrin 5440:Avidin 5334:(fFN: 5261:other: 5190:other: 4997:Maspin 4971:SCCA-1 4421:Human 4398:MEROPS 4380:  4372:  4337:  4327:  4294:  4265:  4258:151637 4255:  4222:  4215:151639 4212:  4179:  4150:  4121:  4113:  4084:  4055:  4026:  4018:  3992:Nature 3981:  3952:  3944:  3904:  3894:  3863:  3853:  3812:  3802:  3763:  3753:  3714:  3669:11 May 3625:11 May 3588:11 May 3551:11 May 3514:11 May 3477:11 May 3440:11 May 3403:11 May 3233:11 May 3203:  3193:  3170:  3134:  3099:  3058:  3048:  3030:Thorax 3009:  2968:  2960:  2889:  2879:  2840:  2830:  2791:  2781:  2742:  2397:ChEMBL 2274:℞-only 2272: 2259: 2248:  2244:℞-only 2230: 2155:  2078:valine 2067:lysine 1978:copies 1866:serpin 1747:fiber 1697:serpin 1655:fucose 1590:-PI). 1465:. The 1337:serpin 1259:search 1257:PubMed 1044:P07758 1040:Q00897 1021:P01009 913:Entrez 645:BioGPS 615:midgut 232:891968 224:107400 5996:Local 5920:Other 5504:Ricin 5477:Other 5325:Other 5084:Serum 4378:S2CID 4119:S2CID 4024:S2CID 3950:S2CID 3696:Chest 3659:(EMA) 3615:(FDA) 3611:U.S. 3578:(FDA) 3574:U.S. 3541:(FDA) 3537:U.S. 3504:(FDA) 3500:U.S. 3467:(FDA) 3463:U.S. 3430:(FDA) 3426:U.S. 3393:(emc) 2966:S2CID 2424:KB2 ( 1947:. As 1812:, an 1784:liver 1680:by a 1641:107 ( 1569:alpha 1554:gamma 1546:alpha 1477:alpha 1435:liver 1423:lungs 1407:blood 1390:from 1364:alpha 1362:) or 1352:alpha 1327:, or 946:20703 906:Mouse 901:Human 872:Amigo 591:liver 525:blood 497:Mouse 490:Human 437:Start 372:Mouse 336:Start 269:Human 240:20103 5896:XIII 5814:VIII 5022:PEDF 4976:CrmA 4966:MENT 4699:9api 4684:8api 4669:7api 4654:2d26 4639:1qmb 4624:1qlp 4609:1psi 4594:1oph 4579:1oo8 4564:1kct 4549:1iz2 4534:1hp7 4519:1ezx 4504:1d5s 4489:1atu 4396:The 4370:PMID 4335:PMID 4292:PMID 4263:PMID 4220:PMID 4177:PMID 4148:PMID 4111:PMID 4082:PMID 4070:1477 4053:PMID 4016:PMID 3979:PMID 3942:PMID 3902:PMID 3861:PMID 3810:PMID 3761:PMID 3712:PMID 3671:2020 3627:2020 3590:2020 3553:2020 3516:2020 3479:2020 3442:2020 3405:2020 3375:2024 3349:2024 3321:2024 3295:2023 3265:2023 3235:2020 3201:OCLC 3191:ISBN 3168:PMID 3132:PMID 3097:PMID 3056:PMID 3007:PMID 2958:PMID 2921:2022 2887:PMID 2838:PMID 2789:PMID 2740:PMID 2515:3130 2509:2001 2427:PDBe 2377:UNII 2368:none 2125:AHFS 1984:, a 1982:gene 1834:gene 1770:and 1707:and 1600:gene 1598:The 1567:The 1550:beta 1461:and 1372:A1AP 1360:A1PI 1346:. A 1344:gene 1313:A1AT 927:5265 480:Bgee 428:Band 389:Chr. 327:Band 286:Chr. 220:OMIM 177:5IO1 173:4PYW 169:9API 165:8API 161:7API 157:3T1P 153:3NE4 149:3NDF 145:3NDD 141:3DRU 137:3DRM 133:3CWM 129:3CWL 125:2QUG 121:2D26 117:1QMB 113:1QLP 109:1PSI 105:1OPH 101:1OO8 97:1KCT 93:1IZ2 89:1HP7 85:1EZX 81:1D5S 77:1ATU 58:RCSB 55:PDBe 5867:VII 5737:B02 5599:SAA 5594:SAP 4360:doi 4325:PMC 4317:doi 4284:doi 4280:352 4253:PMC 4245:doi 4241:110 4210:PMC 4202:doi 4198:110 4169:doi 4140:doi 4103:doi 4099:321 4074:doi 4045:doi 4041:275 4008:doi 3996:298 3971:doi 3934:doi 3892:PMC 3851:PMC 3841:doi 3800:PMC 3792:doi 3751:PMC 3743:doi 3704:doi 3700:117 3160:doi 3124:doi 3120:309 3087:doi 3083:275 3046:PMC 3038:doi 2997:doi 2950:doi 2877:PMC 2869:doi 2828:PMC 2820:doi 2816:201 2779:PMC 2771:doi 2767:179 2732:doi 2728:102 2549:.65 2547:324 2527:601 2521:514 2450:EPA 2261:POM 2200:WHO 2076:to 2065:to 1991:In 1933:. 1885:PAS 1874:Arg 1872:358 1870:Met 1766:of 1694:kDa 1394:of 1329:AAT 1325:A1A 1303:or 450:End 349:End 252:OMA 228:MGI 48:PDB 6166:: 5854:, 5850:/( 5848:IX 5840:, 5836:, 5832:, 5828:, 5824:, 5820:, 5816:/( 5640:C3 5208:, 5122:, 4376:. 4368:. 4356:42 4354:. 4350:. 4333:. 4323:. 4311:. 4307:. 4290:. 4278:. 4261:. 4251:. 4239:. 4235:. 4218:. 4208:. 4196:. 4192:. 4175:. 4165:30 4163:. 4146:. 4136:30 4134:. 4117:. 4109:. 4097:. 4080:. 4068:. 4051:. 4039:. 4022:. 4014:. 4006:. 3994:. 3977:. 3965:. 3948:. 3940:. 3928:. 3900:. 3888:17 3886:. 3882:. 3859:. 3849:. 3837:14 3835:. 3831:. 3808:. 3798:. 3786:. 3782:. 3759:. 3749:. 3737:. 3733:. 3710:. 3698:. 3654:. 3635:^ 3609:. 3598:^ 3572:. 3561:^ 3535:. 3524:^ 3498:. 3487:^ 3461:. 3450:^ 3424:. 3413:^ 3391:. 3365:. 3337:. 3311:. 3281:. 3251:. 3221:. 3199:. 3166:. 3156:80 3154:. 3130:. 3118:. 3095:. 3081:. 3077:. 3054:. 3044:. 3034:59 3032:. 3028:. 3005:. 2993:30 2991:. 2987:. 2964:. 2956:. 2944:. 2932:^ 2923:. 2907:. 2885:. 2875:. 2865:44 2863:. 2859:. 2836:. 2826:. 2814:. 2810:. 2787:. 2777:. 2765:. 2761:. 2738:. 2726:. 2707:. 2689:. 2660:^ 2639:^ 2545:44 2533:10 2430:, 2280:EU 2268:US 2255:UK 2239:CA 2232:S4 2226:AU 2152:US 1960:pH 1880:. 1840:. 1774:. 1751:. 1684:. 1586:(α 1564:. 1449:. 1402:. 1323:, 1321:AT 1315:, 874:/ 456:bp 443:bp 355:bp 342:bp 250:; 246:: 242:; 238:: 234:; 230:: 226:; 222:: 175:, 171:, 167:, 163:, 159:, 155:, 151:, 147:, 143:, 139:, 135:, 131:, 127:, 123:, 119:, 115:, 111:, 107:, 103:, 99:, 95:, 91:, 87:, 83:, 79:, 6142:: 6020:/ 5898:/ 5881:X 5869:/ 5858:) 5844:) 5789:) 5787:3 5780:) 5778:1 5753:) 5749:( 5739:) 5735:( 5725:e 5718:t 5711:v 5570:e 5563:t 5556:v 5495:) 5491:( 5344:/ 5338:) 5237:: 5212:) 5201:) 5176:) 5169:) 5158:: 5146:) 5142:( 5136:) 5132:( 5126:) 5118:( 5108:: 5068:e 5061:t 5054:v 4735:e 4728:t 4721:v 4464:e 4457:t 4450:v 4437:. 4384:. 4362:: 4341:. 4319:: 4313:5 4298:. 4286:: 4269:. 4247:: 4226:. 4204:: 4183:. 4171:: 4154:. 4142:: 4125:. 4105:: 4088:. 4076:: 4059:. 4047:: 4030:. 4010:: 4002:: 3985:. 3973:: 3967:5 3956:. 3936:: 3930:9 3908:. 3867:. 3843:: 3816:. 3794:: 3788:7 3767:. 3745:: 3739:6 3718:. 3706:: 3684:. 3673:. 3629:. 3592:. 3555:. 3518:. 3481:. 3444:. 3407:. 3377:. 3351:. 3323:. 3297:. 3267:. 3237:. 3207:. 3174:. 3162:: 3138:. 3126:: 3103:. 3089:: 3062:. 3040:: 3013:. 2999:: 2972:. 2952:: 2946:6 2893:. 2871:: 2844:. 2822:: 2795:. 2773:: 2746:. 2734:: 2711:. 2693:. 2530:S 2524:O 2518:N 2512:H 2506:C 2452:) 2448:( 2436:) 2282:: 2270:: 2257:: 2228:: 2127:/ 2058:) 2006:i 2004:P 1999:i 1997:P 1967:M 1854:i 1852:P 1847:i 1845:P 1830:1 1717:i 1715:P 1713:( 1611:1 1588:1 1556:( 1479:1 1370:( 1366:1 1358:( 1354:1 1319:1 1317:α 1311:( 1307:1 1305:α 374:) 271:) 254::

Index


PDB
PDBe
RCSB
1ATU
1D5S
1EZX
1HP7
1IZ2
1KCT
1OO8
1OPH
1PSI
1QLP
1QMB
2D26
2QUG
3CWL
3CWM
3DRM
3DRU
3NDD
3NDF
3NE4
3T1P
7API
8API
9API
4PYW
5IO1

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