29:
164:
These infections are more often caused by gram-negative bacteria (such as
Neisseria meningitidis, Haemophilus influenzae, Klebsiella aerogenes, and Escherichia coli), but can also be caused by gram-positive bacteria (such as Streptococcus pneumoniae, Streptoccocus pyogenes, Staphylococcus aureus, and
151:
In addition to studying C3 deficiency in diagnosed human patients, researchers have studied C3 deficiency in animals. C3 deficiency can be induced by injecting animals with cobra venom factor, which functions like an unregulated C3-convertase because factor I and factor H do not regulate it, cleaving
118:
The human gene for C3 is located on chromosome 19. In a healthy individual, C3 is secreted by hepatocytes (liver cells) as well as monocytes and macrophages, dendritic cells, polymorphonuclear leukocytes, fibroblasts, and endothelial cells. These cells will increase production of C3 in response to
94:
immune response. C3 is one of over 30 complement proteins circulating in the blood. C3 circulates in an inactive form but can be activated in order to aid the immune system's response to a foreign invader. C3 is the most abundant complement component and is a particularly important complement
176:. (Note that some studies have grouped rheumatic and renal diseases under the broader category of immune complex-mediated diseases.) There have been recorded cases of C3 deficient patients that suffer only from immune complex-mediated diseases and not severe, recurrent infections.
147:
As of 2016, primary C3 deficiency had been reported in 37 individuals (from 29 families) around the world. Patients with primary or secondary C3 deficiency generally displayed symptoms (i.e. suffered from infection) within the first 2 years of life.
67:(C3). People can suffer from either primary or secondary C3 deficiency. Primary C3 deficiency refers to an inherited autosomal-recessive disorder that involves mutations in the gene for C3. Secondary C3 deficiency results from a lack of
623:
115:, which will cleave C3 molecules into C3a and C3b fragments. This is important because an unregulated C3-convertase will cause a deficiency of intact C3 molecules by generating these fragments.
616:
609:
210:
Okura, Yuka; Kobayashi, Ichiro; Yamada, Masafumi; Sasaki, Satoshi; Yamada, Yutaka; Kamioka, Ichiro; Kanai, Rie; Takahashi, Yutaka; Ariga, Tadashi (2016).
841:
689:
878:
173:
75:, two proteins that are key for the regulation of C3. Both primary and secondary C3 deficiency are characterized by low levels or absence of C3.
1005:
324:
Skattum, Lillemor; van Deuren, Marcel; van der Poll, Tom; Truedsson, Lennart (2011). "Complement deficiency states and associated infections".
707:
478:
446:
388:"Clinical Aspects and Molecular Basis of Primary Deficiencies of Complement Component C3 and its Regulatory Proteins Factor I and Factor H"
1010:
562:
168:
In some cases, primary and secondary C3 deficiency have been associated with the onset of rheumatic or renal (kidney) diseases, such as
165:
Streptococcus milleri). Infections of the respiratory tract, such as pneumonia, have commonly been observed in C3 deficient patients.
945:
862:
773:
1036:
1015:
885:
757:
104:
179:
Treatment for C3 deficiency has generally involved the prescription of regular antibiotics intended to prevent infection.
752:
169:
100:
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851:
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648:
573:
601:
985:
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91:
83:
64:
37:
975:
955:
676:
504:"Molecular analysis of hereditary deficiency of the third component of complement (C3) in two sisters"
995:
699:
120:
87:
152:
most C3 molecules in the serum into C3a and C3b fragments, which depletes the amount of intact C3.
68:
847:
722:
136:
816:
807:
792:
636:
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436:
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49:
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937:
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712:
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108:
1030:
520:
503:
404:
387:
124:
112:
99:, but C3 plays a central role in all three of these pathways (see the pages for the
966:
778:
502:
Matsuyama W, Nakagawa M, Takashima H, Muranaga F, Sano Y, Osame M (December 2001).
160:
C3 deficiency makes an individual susceptible to severe, recurrent infections from
28:
578:
337:
473:. Bartlett, John G., Blacklow, Neil R. (3rd ed.). Philadelphia: LWW (PE).
279:
228:
212:"Clinical characteristics and genotype-phenotype correlations in C3 deficiency"
211:
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413:
345:
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139:, which is expressed in the outer membrane of gram-negative bacteria.
797:
666:
41:
567:
605:
264:"Disease-Causing Mutations in Genes of the Complement System"
262:
Degn, Søren E.; Jensenius, Jens C.; Thiel, Steffen (2011).
95:
component because there are three ways to activate the
111:). Each of these pathways involves the formation of a
544:
954:
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861:
791:
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675:
656:
647:
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48:
21:
386:S. Reis, E.; Falcao, D. A.; Isaac, L. (2006).
617:
8:
842:Purine nucleoside phosphorylase deficiency
690:Transient hypogammaglobulinemia of infancy
672:
653:
624:
610:
602:
545:
216:Journal of Allergy and Clinical Immunology
27:
18:
519:
438:Textbook of Microbiology & Immunology
403:
295:
227:
174:membranoproliferative glomerulonephritis
191:
86:(C3) is a protein involved in both the
1006:Terminal complement pathway deficiency
268:The American Journal of Human Genetics
7:
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135:) or in response to the presence of
1011:Paroxysmal nocturnal hemoglobinuria
392:Scandinavian Journal of Immunology
14:
441:. Elsevier India. pp. 125–.
63:is a genetic condition affecting
774:Common variable immunodeficiency
521:10.2169/internalmedicine.40.1254
405:10.1111/j.1365-3083.2006.01729.x
143:Cases of complement 3 deficiency
946:Idiopathic CD4+ lymphocytopenia
1016:Complement receptor deficiency
886:Adenosine deaminase deficiency
1:
469:Gorbach, Sherwood L. (2003).
338:10.1016/j.molimm.2011.05.001
170:systemic lupus erythematosus
685:X-linked agammaglobulinemia
1053:
280:10.1016/j.ajhg.2011.05.011
229:10.1016/j.jaci.2015.08.017
119:cytokine signaling (from
35:
26:
901:Bare lymphocyte syndrome
753:Wiskott–Aldrich syndrome
1001:Complement 3 deficiency
986:Complement 4 deficiency
982:Complement 2 deficiency
61:Complement 3 deficiency
22:Complement 3 deficiency
162:encapsulated bacteria.
84:Complement component 3
65:complement component 3
1037:Complement deficiency
976:Hereditary angioedema
832:Ataxia–telangiectasia
677:Hypogammaglobulinemia
435:Parija (2009-01-01).
156:Clinical implications
44:rendering of PDB 1c3d
996:Properdin deficiency
817:Di George's syndrome
700:Dysgammaglobulinemia
326:Molecular Immunology
471:Infectious Diseases
105:alternative pathway
848:Hyper IgM syndrome
758:Hyper-IgE syndrome
723:Hyper IgM syndrome
639:disorders causing
137:lipopolysaccharide
1024:
1023:
909:
908:
808:thymic hypoplasia
793:T cell deficiency
787:
786:
599:
598:
480:978-1-4698-7418-0
448:978-81-312-2163-1
332:(14): 1643–1655.
222:(2): 640–644.e1.
101:classical pathway
97:complement system
58:
57:
36:Structure of the
16:Medical condition
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896:ZAP70 deficiency
827:Nezelof syndrome
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641:immunodeficiency
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133:interferon gamma
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938:Lymphocytopenia
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991:MBL deficiency
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891:Omenn syndrome
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718:IgM deficiency
715:
713:IgG deficiency
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708:IgA deficiency
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549:Classification
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541:External links
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514:(12): 1254–8.
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398:(3): 155–168.
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274:(6): 689–705.
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109:lectin pathway
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823:euparathyroid
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113:C3-convertase
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967:C1-inhibitor
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779:ICF syndrome
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452:. Retrieved
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839:peripheral:
508:Intern. Med
454:13 November
79:C3 function
40:. Based on
972:Angioedema
958:deficiency
956:Complement
933:Leukopenia
883:autosomal:
637:complement
585:DiseasesDB
186:References
107:, and the
54:Immunology
38:C3 protein
876:x-linked:
489:923530065
414:0300-9475
346:0161-5890
288:0002-9297
238:0091-6749
129:TNF-alpha
50:Specialty
1031:Category
923:HIV/AIDS
915:Acquired
866:combined
658:Antibody
633:Lymphoid
530:11813855
422:16499568
354:21624663
306:21664996
246:26435005
92:adaptive
73:factor H
69:factor I
662:humoral
649:Primary
579:C565169
297:3113252
879:X-SCID
863:Severe
568:120700
528:
487:
477:
445:
420:
412:
352:
344:
304:
294:
286:
244:
236:
88:innate
869:(B+T)
767:Other
131:, or
42:PyMOL
635:and
590:1869
574:MeSH
563:OMIM
526:PMID
485:OCLC
475:ISBN
456:2010
443:ISBN
418:PMID
410:ISSN
350:PMID
342:ISSN
302:PMID
284:ISSN
242:PMID
234:ISSN
172:and
125:IL-6
121:IL-1
90:and
516:doi
400:doi
334:doi
292:PMC
276:doi
224:doi
220:137
71:or
1033::
588::
577::
566::
524:.
512:40
510:.
506:.
483:.
416:.
408:.
396:63
394:.
390:.
362:^
348:.
340:.
330:48
328:.
314:^
300:.
290:.
282:.
272:88
270:.
266:.
254:^
240:.
232:.
218:.
214:.
194:^
127:,
123:,
103:,
984:/
978:)
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970:(
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834:)
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727:1
725:(
669:)
667:B
665:(
660:/
625:e
618:t
611:v
555:D
532:.
518::
491:.
458:.
424:.
402::
356:.
336::
308:.
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248:.
226::
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