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Hemoglobin C

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209:(HbS) from one parent and the gene for hemoglobin C disease (HbC) from the other parent. Since HbC does not polymerize as readily as HbS, there is less sickling in most cases. There are fewer acute vaso-occlusive events and therefore in some cases fewer sickle cell crises. The peripheral smear demonstrates mostly target cells, occasional hemoglobin C crystals, and only a few sickle cells. However, persons with hemoglobin SC disease (HbSC) have more significant retinopathy, ischemic necrosis of bone, and priapism than those with pure SS disease. 50: 194: 493: 429:
discovered from two African-American families a different blood condition very similar to sickle cell disease. Five of the ten individuals indicated sickled RBCs. But the condition was harmless as the individuals had no anaemia. Thus, it was not clear whether it was involved in sickle cell disease.
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or hemoglobin S. Hemoglobin C mutation is an autosomal recessive disorder that results from the biparental inheritance of the allele that encodes for hemoglobin C. If both parents are carriers of hemoglobin C, there is a chance of having a child with hemoglobin C disease. Assuming both parents are
309:. Evidences indicate that HbC reduced the level of PfEMP1, which is required for effective binding and invasion of RBC by the malarial parasite. It has been predicted that with the trend of HbC mutation and falciparum prevalence, HbC would replace HbS in central West Africa in the future. 364:. Mild-to-moderate reduction in RBC lifespan may accompany from mild hemolytic anemia. Individuals with hemoglobin C disease have sporadic episodes of musculoskeletal (joint) pain. People with hemoglobin C disease can expect to lead a normal life. 1751: 1736: 252:
carriers, there is a 25% chance of having a child with hemoglobin C disease, a 50% chance of having a child who is a carrier of hemoglobin C, and a 25% chance of having a child who is neither a carrier nor affected by hemoglobin C disease.
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Fairhurst, R. M.; Fujioka, H.; Hayton, K.; Collins, K. F.; Wellems, T. E. (2003). "Aberrant development of Plasmodium falciparum in hemoglobin CC red cells: implications for the malaria protective effect of the homozygous state".
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Fairhurst, Rick M.; Baruch, Dror I.; Brittain, Nathaniel J.; Ostera, Graciela R.; Wallach, John S.; Hoang, Holly L.; Hayton, Karen; Guindo, Aldiouma; Makobongo, Morris O.; Schwartz, Owen M.; Tounkara, Anatole (2005).
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and is less effective in resistance to falciparum malaria in heterozygous conditions. Homozygous HbC is more resistant to heterozygous condition, or to thalassemias. But HbC mutation does not prevent the infection.
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chain of the hemoglobin. The mutation can be homozygous, occurring on both the chromosomes (alleles), or heterozygous, affecting only one allele. Under heterozygous condition, people are said to have hemoglobin C
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malaria infection. HbC has been described as being more advantageous than HbS because, even in homozygous individuals, it is usually non-fatal. However, in contrast to HbS, it does not prevent malaria due to
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while 8% of African-Americans have hemoglobin S (Sickle) gene. Thus Hemoglobin SC disease is significantly more common than Hemoglobin CC disease. The trait also affects people whose ancestors came from
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Travassos, Mark A.; Coulibaly, Drissa; Laurens, Matthew B.; Dembélé, Ahmadou; Tolo, Youssouf; Koné, Abdoulaye K.; Traoré, Karim; Niangaly, Amadou; Guindo, Aldiouma; Wu, Yukun; Berry, Andrea A. (2015).
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Kreuels, Benno; Kreuzberg, Christina; Kobbe, Robin; Ayim-Akonor, Matilda; Apiah-Thompson, Peter; Thompson, Benedicta; Ehmen, Christa; Adjei, Samuel; Langefeld, Iris; Adjei, Ohene; May, Jürgen (2010).
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region. However, it is possible for a person of any race or nationality to have hemoglobin C trait. In terms of geographic distribution, the hemoglobin C allele is found at the highest frequencies in
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gene. People with one copy of the gene for hemoglobin C do not experience symptoms, but can pass the abnormal gene on to their children. Those with two copies of the gene are said to have
137:) do not experience significant symptoms, but can pass the abnormal gene onto their children; this condition is called hemoglobin C trait. When two hemoglobin C genes are present (termed 115:
in 1950 in two African-American families. It has since been established that it is most common among people in West Africa. It confers survival benefits as individuals with HbC are
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The hemoglobin was named hemoglobin III, but hemoglobin C was eventually used. By 1954, it was found that the mutant hemoglobin was highly prevalent in West Africa. In 1960,
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do not survive in red blood cells with homozygous hemoglobins, but can survive in the presence of heterozygous hemoglobins. HbC reduced the binding force (cytoadherence) of
2532: 247:, or as hemoglobin C carriers, and they have one gene for HbC with either one HbA gene or HbS gene. Their red blood cells contain both hemoglobin C and either normal 978:"Abnormal PfEMP1/knob display on Plasmodium falciparum-infected erythrocytes containing hemoglobin variants: fresh insights into malaria pathogenesis and protection" 205:
HbC can combine with other abnormal hemoglobins and cause serious hemoglobinopathies. Individuals with sickle cell–hemoglobin C (HbSC), have inherited the gene for
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Modiano, D.; Luoni, G.; Sirima, B. S.; Simporé, J.; Verra, F.; Konaté, A.; Rastrelli, E.; Olivieri, A.; Calissano, C.; Paganotti, G. M.; D'Urbano, L. (2001).
174: 405:. Hemoglobin C disease is present at birth, though some cases may not be diagnosed until adulthood. Both males and females are affected equally. 1851: 441:
and J. A. Hunt at the University of Cambridge discovered that the mutation was a single amino acid replacement of glutamic acid with lysine.
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at the California Institute of Technology discovered in 1949 that the disease was due to abnormal hemoglobin called HBS. In 1950, Itano and
116: 596: 145:, as red blood cells containing hemoglobin C have a decreased lifespan. The anemia in hemoglobin C disease is classified as 434:
condition. The next year, Neel and his colleagues established that the hemoglobin is associated with sickle cell disease.
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may produce pigmented gallstones, an unusual type of gallstone composed of the dark-colored contents of red blood cells.
1540: 1844: 322: 1579: 1541:"SICKLE-CELL-HEMOGLOBIN-C DISEASE: Report of a Case with Electrophoretic Studies of Hemoglobin in Family Members" 2557: 1919: 1766: 1712: 1702: 1997: 1978: 1941: 234: 84: 326: 1837: 193: 121: 770:"Differing effects of HbS and HbC traits on uncomplicated falciparum malaria, anemia, and child growth" 516:
Nagel, Ronald L.; Fabry, Mary E.; Steinberg, Martin H. (2003). "The paradox of hemoglobin SC disease".
1580:"A case of sickle cell — Haemoglobin C disease and a survey of haemoglobin C incidence in West Africa" 2552: 2122: 2108: 2094: 2080: 1370: 1186: 1056: 932: 871:"Haemoglobinopathies and the clinical epidemiology of malaria: a systematic review and meta-analysis" 827: 672:"Haemoglobinopathies and the clinical epidemiology of malaria: a systematic review and meta-analysis" 318: 2388: 2358: 2282: 1924: 1755: 1279:"Date on the occurrence of hemoglobin C and other abnormal hemoglobins in some African populations" 414: 206: 49: 2372: 2054: 1638: 1277:
Hiernauz, J.; Linhard, J.; Livingstone, F. B.; Neel, J. V.; Robinson, A.; Zuelzer, W. W. (1956).
1259: 1210: 1159: 1080: 958: 851: 361: 97: 1708: 1657: 921:"Abnormal display of PfEMP-1 on erythrocytes carrying haemoglobin C may protect against malaria" 96:. It is possible for a person to have both the gene for hemoglobin S (the form associated with 2353: 2248: 1806: 1777: 1677: 1599: 1560: 1488: 1439: 1398: 1339: 1300: 1251: 1202: 1151: 1116: 1072: 1007: 950: 900: 843: 791: 750: 701: 571: 533: 476: 104:, and is generally more severe than hemoglobin C disease, but milder than sickle cell anemia. 38: 1134:
Edington, G. M.; Lehmann, H. (1956). "36. The Distribution of Haemoglobin C in West Africa".
2258: 2217: 2147: 1829: 1669: 1630: 1591: 1552: 1519: 1478: 1470: 1429: 1388: 1378: 1331: 1290: 1241: 1194: 1143: 1108: 1064: 997: 989: 940: 890: 882: 835: 781: 740: 732: 721:"Hemoglobin C Trait Provides Protection From Clinical Falciparum Malaria in Malian Children" 691: 683: 563: 525: 377: 349:
supplementation may help produce normal red blood cells and improve the symptoms of anemia
276: 260: 244: 221: 146: 1418:"A new inherited abnormality of hemoglobin and its interaction with sickle cell hemoglobin" 2512: 2367: 497: 492: 238:
gene causes amino acid substitution of glutamic acid to lysine at the 6th position of the
1817: 1556: 650: 1698: 1374: 1335: 1190: 1060: 936: 831: 620: 177:(MCHC). The high MCHC is caused by a decreased concentration of water inside the cells. 2414: 2409: 1483: 1458: 1393: 1358: 1295: 1278: 1068: 1002: 977: 895: 870: 745: 720: 696: 671: 1760: 886: 687: 529: 372:
Hemoglobin C is found most abundantly in areas of West Africa, such as Nigeria, where
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The red blood cells of people with hemoglobin C disease are usually abnormally small (
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Genetic counseling may be appropriate for high-risk couples who wish to have a baby.
153:, and sometimes jaundice, may also occur. Some persons with this disease may develop 112: 71: 17: 1263: 1214: 1084: 2507: 2448: 2324: 2310: 2186: 2161: 962: 855: 422: 256: 248: 217: 213: 150: 134: 108: 1524: 1434: 1417: 1035: 317:
Physical examination may show an enlarged spleen. Tests that may be done include:
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Fairhurst, Rick M.; Casella, James F. (2004). "Homozygous Hemoglobin C Disease".
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Proceedings of the National Academy of Sciences of the United States of America
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Pauling L, Itano HA (November 1949). "Sickle cell anemia a molecular disease".
1112: 468: 181:, microspherocytes, and HbC crystals can be seen on microscopic examination of 2502: 2460: 2363: 1886: 1745: 1319: 346: 170: 154: 138: 67: 43: 1155: 1047:
Akinyanju, Olufemi O. (1989). "A Profile of Sickle Cell Disease in Nigeria".
141:), the individual is said to have hemoglobin C disease, and may develop mild 2443: 1812: 736: 655: 394: 158: 1681: 1603: 1564: 1492: 1443: 1402: 1383: 1304: 1255: 1206: 1120: 1011: 954: 904: 847: 815: 795: 754: 705: 575: 537: 480: 1343: 1076: 567: 431: 272: 264: 178: 1474: 945: 920: 1860: 1642: 1618: 1163: 816:"Haemoglobin C protects against clinical Plasmodium falciparum malaria" 621:"Updating PubMed Health - National Library of Medicine - PubMed Health" 402: 373: 267:
for the mutation, about 28–44% of total hemoglobin (Hb) is HbC, and no
1728: 1459:"A review of the varieties of human haemoglobin in health and disease" 1877: 1740: 839: 386: 358: 306: 268: 142: 93: 79: 1634: 1147: 869:
Taylor, Steve M.; Parobek, Christian M.; Fairhurst, Rick M. (2012).
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Taylor, Steve M.; Parobek, Christian M.; Fairhurst, Rick M. (2012).
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Transactions of the Royal Society of Tropical Medicine and Hygiene
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Fairhurst, Rick M.; Bess, Cameron D.; Krause, Michael A. (2012).
149:, because it is caused by the destruction of red blood cells. An 2038: 2026: 2021: 1953: 1864: 1833: 2268: 2009: 239: 75: 133:
People with one copy of the gene for hemoglobin C (termed
496: This article incorporates text available under the 255:
This mutated form reduces the normal plasticity of host
1320:"Diseases due to abnormalities of hemoglobin structure" 401:, where it has been associated with protection against 275:, nearly all Hb is in the HbC form, resulting in mild 232:
Hemoglobin C is produced when a point mutation in the
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There are also a few cases of HbC in combination with
100:) and the gene for hemoglobin C; this state is called 467:
Karna, Bibek; Jha, Suman K.; Al Zaabi, Eiman (2020),
1718: 2495: 2469: 2434: 2427: 2402: 2381: 2346: 2239: 2179: 2140: 2073: 2062: 2053: 1976: 1901: 1894: 1885: 1876: 1792: 1722: 37: 32: 1619:"The Distribution of Haemoglobin C in West Africa" 197:Blood film of hemoglobin SC disease, showing many 1359:"A new inherited abnormality of human hemoglobin" 430:Genetically, the abnormal hemoglobin was only in 357:Overall, hemoglobin C disease is one of the more 1416:Kaplan, E.; Zuelzer, W. W.; Neel, J. V. (1951). 475:, Treasure Island (FL): StatPearls Publishing, 1845: 8: 376:live. Hemoglobin C gene is found in 2-3% of 511: 509: 507: 2431: 2070: 2059: 1898: 1891: 1882: 1852: 1838: 1830: 1719: 1230:"One hundred years of sickle cell disease" 1049:Annals of the New York Academy of Sciences 287:Individuals with HbC have reduced risk of 48: 29: 2533:disorders of globin and globulin proteins 1711:at the U.S. National Library of Medicine 1701:at the U.S. National Library of Medicine 1523: 1482: 1433: 1392: 1382: 1294: 1245: 1001: 944: 894: 785: 744: 695: 175:mean corpuscular hemoglobin concentration 450: 82:residue due to a point mutation in the 1545:A.M.A. Journal of Diseases of Children 1025: 1023: 1021: 279:, jaundice and enlargement of spleen. 1617:Edington, G. M.; Lehmann, H. (1956). 809: 807: 805: 645: 643: 641: 639: 637: 615: 613: 549: 547: 7: 1557:10.1001/archpedi.1956.02060020040008 1457:White, J. C.; Beaver, G. H. (1954). 665: 663: 591: 589: 587: 585: 462: 460: 458: 456: 454: 1506:Singer, Karl; Singer, Lily (1953). 1336:10.1146/annurev.me.21.020170.001105 74:residue at the 6th position of the 1578:Edington, G.M; Lehmann, H (1954). 1357:Itano, H. A.; Neel, J. V. (1950). 1283:American Journal of Human Genetics 1069:10.1111/j.1749-6632.1989.tb24159.x 725:The Journal of Infectious Diseases 189:Combinations with other conditions 157:that require treatment. Continued 25: 1656:Hunt, J.A.; Ingram, V.M. (1960). 1508:"Studies on Abnormal Hemoglobins" 1247:10.1111/j.1365-2141.2010.08419.x 491: 413:Studying the molecular basis of 345:Usually no treatment is needed. 556:New England Journal of Medicine 1234:British Journal of Haematology 875:The Lancet Infectious Diseases 623:. Ncbi.nlm.nih.gov. 2014-11-12 117:naturally resistant to malaria 1: 1662:Biochimica et Biophysica Acta 1658:"Abnormal human haemoglobins" 1525:10.1182/blood.V8.11.1008.1008 1463:Journal of Clinical Pathology 1435:10.1182/blood.V6.12.1240.1240 1228:Serjeant GR (December 2010). 887:10.1016/S1473-3099(12)70055-5 688:10.1016/S1473-3099(12)70055-5 530:10.1016/S0268-960X(03)00003-1 1674:10.1016/0006-3002(60)90818-0 1596:10.1016/0035-9203(54)90104-2 1199:10.1126/science.110.2865.543 994:10.1016/j.micinf.2012.05.006 787:10.1182/blood-2009-09-241844 305:by reducing the activity of 165:Red blood cell abnormalities 2574: 1539:Schell, Norman B. (1956). 1113:10.1182/blood-2002-10-3105 323:hemoglobin electrophoresis 185:from homozygous patients. 2528: 1324:Annual Review of Medicine 78:chain is replaced with a 1713:Medical Subject Headings 1703:Medical Subject Headings 1031:MedlinePlus Encyclopedia 125:, albeit incompletely. 92:and can experience mild 1384:10.1073/pnas.36.11.613 1318:Huehns, E. R. (1970). 982:Microbes and Infection 469:"Hemoglobin C Disease" 327:peripheral blood smear 202: 107:HbC was discovered by 737:10.1093/infdis/jiv308 568:10.1056/NEJMicm030486 283:Resistance to malaria 196: 122:Plasmodium falciparum 102:hemoglobin SC disease 18:Hemoglobin SC disease 1709:Hemoglobin+C+Disease 1036:Hemoglobin C disease 651:Hemoglobin C Disease 597:"Hemoglobin C Trait" 319:complete blood count 201:and few sickle cells 90:hemoglobin C disease 2389:Glycated hemoglobin 2359:Carbaminohemoglobin 1475:10.1136/jcp.7.3.175 1375:1950PNAS...36..613I 1191:1949Sci...110..543P 1061:1989NYASA.565..126A 946:10.1038/nature03631 937:2005Natur.435.1117F 931:(7045): 1117–1121. 832:2001Natur.414..305M 415:sickle cell disease 263:. In those who are 207:sickle cell disease 1793:External resources 362:hemoglobinopathies 203: 129:Symptoms and signs 98:sickle cell anemia 2540: 2539: 2491: 2490: 2487: 2486: 2423: 2422: 2354:Carboxyhemoglobin 2342: 2341: 2235: 2234: 2049: 2048: 1827: 1826: 1518:(11): 1008–1023. 1428:(12): 1240–1249. 826:(6861): 305–308. 780:(22): 4551–4558. 731:(11): 1778–1786. 378:African-Americans 66:) is an abnormal 57: 56: 27:Medical condition 16:(Redirected from 2565: 2432: 2071: 2060: 1899: 1892: 1883: 1854: 1847: 1840: 1831: 1720: 1686: 1685: 1653: 1647: 1646: 1614: 1608: 1607: 1575: 1569: 1568: 1536: 1530: 1529: 1527: 1503: 1497: 1496: 1486: 1454: 1448: 1447: 1437: 1413: 1407: 1406: 1396: 1386: 1354: 1348: 1347: 1315: 1309: 1308: 1298: 1274: 1268: 1267: 1249: 1225: 1219: 1218: 1174: 1168: 1167: 1131: 1125: 1124: 1095: 1089: 1088: 1044: 1038: 1027: 1016: 1015: 1005: 973: 967: 966: 948: 915: 909: 908: 898: 866: 860: 859: 840:10.1038/35104556 811: 800: 799: 789: 765: 759: 758: 748: 716: 710: 709: 699: 667: 658: 647: 632: 631: 629: 628: 617: 608: 607: 605: 604: 593: 580: 579: 551: 542: 541: 513: 502: 495: 489: 488: 487: 464: 277:hemolytic anemia 261:hemoglobinopathy 222:beta thalassemia 62:(abbreviated as 53: 52: 30: 21: 2573: 2572: 2568: 2567: 2566: 2564: 2563: 2562: 2558:Blood disorders 2543: 2542: 2541: 2536: 2524: 2513:Cytochrome P450 2483: 2465: 2419: 2398: 2377: 2368:Deoxyhemoglobin 2338: 2334: 2330: 2320: 2316: 2306: 2302: 2292: 2288: 2278: 2274: 2264: 2254: 2231: 2227: 2223: 2213: 2209: 2204: 2196: 2192: 2175: 2171: 2167: 2157: 2153: 2136: 2132: 2128: 2123:HbE Portland II 2118: 2114: 2104: 2100: 2090: 2086: 2065: 2045: 1972: 1903:Alpha locus on 1872: 1858: 1828: 1823: 1822: 1788: 1787: 1731: 1695: 1690: 1689: 1655: 1654: 1650: 1635:10.2307/2793520 1616: 1615: 1611: 1577: 1576: 1572: 1538: 1537: 1533: 1505: 1504: 1500: 1456: 1455: 1451: 1415: 1414: 1410: 1369:(11): 613–617. 1356: 1355: 1351: 1317: 1316: 1312: 1276: 1275: 1271: 1227: 1226: 1222: 1185:(2865): 543–8. 1176: 1175: 1171: 1148:10.2307/2793520 1133: 1132: 1128: 1097: 1096: 1092: 1046: 1045: 1041: 1028: 1019: 988:(10): 851–862. 975: 974: 970: 917: 916: 912: 868: 867: 863: 813: 812: 803: 767: 766: 762: 718: 717: 713: 669: 668: 661: 648: 635: 626: 624: 619: 618: 611: 602: 600: 595: 594: 583: 553: 552: 545: 515: 514: 505: 485: 483: 466: 465: 452: 447: 411: 370: 355: 343: 335: 315: 285: 230: 191: 167: 151:enlarged spleen 131: 47: 28: 23: 22: 15: 12: 11: 5: 2571: 2569: 2561: 2560: 2555: 2545: 2544: 2538: 2537: 2529: 2526: 2525: 2523: 2522: 2517: 2516: 2515: 2510: 2499: 2497: 2493: 2492: 2489: 2488: 2485: 2484: 2482: 2481: 2475: 2473: 2467: 2466: 2464: 2463: 2458: 2453: 2452: 2451: 2440: 2438: 2429: 2425: 2424: 2421: 2420: 2418: 2417: 2415:Erythrocruorin 2412: 2406: 2404: 2400: 2399: 2397: 2396: 2391: 2385: 2383: 2379: 2378: 2376: 2375: 2373:Sulfhemoglobin 2370: 2361: 2356: 2350: 2348: 2344: 2343: 2340: 2339: 2337: 2336: 2332: 2328: 2322: 2318: 2314: 2308: 2304: 2300: 2294: 2290: 2286: 2280: 2276: 2272: 2266: 2262: 2256: 2252: 2245: 2243: 2237: 2236: 2233: 2232: 2230: 2229: 2225: 2221: 2215: 2211: 2207: 2202: 2198: 2194: 2190: 2183: 2181: 2177: 2176: 2174: 2173: 2169: 2165: 2159: 2155: 2151: 2144: 2142: 2138: 2137: 2135: 2134: 2130: 2126: 2120: 2116: 2112: 2109:HbE Portland I 2106: 2102: 2098: 2092: 2088: 2084: 2077: 2075: 2068: 2057: 2051: 2050: 2047: 2046: 2044: 2043: 2042: 2041: 2031: 2030: 2029: 2024: 2014: 2013: 2012: 2002: 2001: 2000: 1989: 1987: 1974: 1973: 1971: 1970: 1969: 1968: 1958: 1957: 1956: 1946: 1945: 1944: 1934: 1933: 1932: 1927: 1922: 1911: 1909: 1896: 1889: 1880: 1874: 1873: 1859: 1857: 1856: 1849: 1842: 1834: 1825: 1824: 1821: 1820: 1818:article/200853 1809: 1797: 1796: 1794: 1790: 1789: 1786: 1785: 1774: 1763: 1748: 1732: 1727: 1726: 1724: 1723:Classification 1717: 1716: 1706: 1694: 1693:External links 1691: 1688: 1687: 1648: 1609: 1590:(4): 332–336. 1570: 1531: 1498: 1469:(3): 175–200. 1449: 1408: 1349: 1310: 1289:(3): 138–150. 1269: 1220: 1169: 1126: 1107:(8): 3309–15. 1090: 1039: 1017: 968: 910: 881:(6): 457–468. 861: 801: 760: 711: 682:(6): 457–468. 659: 633: 609: 581: 543: 503: 449: 448: 446: 443: 410: 407: 369: 366: 354: 351: 342: 339: 334: 331: 314: 311: 284: 281: 229: 226: 190: 187: 173:) with a high 166: 163: 130: 127: 55: 54: 41: 35: 34: 26: 24: 14: 13: 10: 9: 6: 4: 3: 2: 2570: 2559: 2556: 2554: 2551: 2550: 2548: 2535: 2534: 2527: 2521: 2520:Methemalbumin 2518: 2514: 2511: 2509: 2506: 2505: 2504: 2501: 2500: 2498: 2494: 2480: 2479:Leghemoglobin 2477: 2476: 2474: 2472: 2468: 2462: 2459: 2457: 2454: 2450: 2447: 2446: 2445: 2442: 2441: 2439: 2437: 2433: 2430: 2426: 2416: 2413: 2411: 2410:Chlorocruorin 2408: 2407: 2405: 2401: 2395: 2394:Methemoglobin 2392: 2390: 2387: 2386: 2384: 2380: 2374: 2371: 2369: 2365: 2364:Oxyhemoglobin 2362: 2360: 2357: 2355: 2352: 2351: 2349: 2345: 2326: 2323: 2312: 2309: 2298: 2295: 2284: 2281: 2270: 2267: 2260: 2257: 2250: 2247: 2246: 2244: 2242: 2238: 2219: 2216: 2205: 2199: 2188: 2185: 2184: 2182: 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853: 849: 845: 841: 837: 833: 829: 825: 821: 817: 810: 808: 806: 802: 797: 793: 788: 783: 779: 775: 771: 764: 761: 756: 752: 747: 742: 738: 734: 730: 726: 722: 715: 712: 707: 703: 698: 693: 689: 685: 681: 677: 673: 666: 664: 660: 657: 653: 652: 646: 644: 642: 640: 638: 634: 622: 616: 614: 610: 598: 592: 590: 588: 586: 582: 577: 573: 569: 565: 561: 557: 550: 548: 544: 539: 535: 531: 527: 524:(3): 167–78. 523: 519: 518:Blood Reviews 512: 510: 508: 504: 501: 499: 494: 482: 478: 474: 470: 463: 461: 459: 457: 455: 451: 444: 442: 440: 439:Vernon Ingram 435: 433: 428: 427:James V. Neel 424: 420: 419:Linus Pauling 416: 408: 406: 404: 400: 396: 392: 391:Latin America 388: 384: 379: 375: 367: 365: 363: 360: 352: 350: 348: 340: 338: 332: 330: 328: 324: 320: 312: 310: 308: 304: 303:P. falciparum 300: 299:P. falciparum 295: 290: 289:P. falciparum 282: 280: 278: 274: 271:develops. In 270: 266: 262: 258: 253: 250: 246: 241: 237: 236: 227: 225: 223: 219: 215: 210: 208: 200: 195: 188: 186: 184: 180: 176: 172: 164: 162: 160: 156: 152: 148: 144: 140: 136: 128: 126: 124: 123: 118: 114: 113:James V. Neel 110: 105: 103: 99: 95: 91: 87: 86: 81: 77: 73: 72:glutamic acid 69: 65: 61: 51: 45: 42: 40: 36: 31: 19: 2530: 2508:Cytochrome b 2470: 2449:Metmyoglobin 2435: 2296: 2240: 2066:development: 2063: 2033: 2016: 2004: 1992: 1977: 1960: 1948: 1936: 1914: 1902: 1869:hemoproteins 1811: 1800: 1776: 1765: 1750: 1735: 1699:Hemoglobin+C 1665: 1661: 1651: 1626: 1622: 1612: 1587: 1583: 1573: 1551:(1): 38–44. 1548: 1544: 1534: 1515: 1511: 1501: 1466: 1462: 1452: 1425: 1421: 1411: 1366: 1362: 1352: 1327: 1323: 1313: 1286: 1282: 1272: 1240:(5): 425–9. 1237: 1233: 1223: 1182: 1178: 1172: 1139: 1135: 1129: 1104: 1100: 1093: 1052: 1048: 1042: 1029: 985: 981: 971: 928: 924: 913: 878: 874: 864: 823: 819: 777: 773: 763: 728: 724: 714: 679: 675: 649: 625:. Retrieved 601:. Retrieved 599:. Stjude.org 559: 555: 521: 517: 490: 484:, retrieved 472: 436: 432:heterozygous 423:Harvey Itano 412: 371: 368:Epidemiology 356: 344: 336: 316: 302: 298: 293: 288: 286: 265:heterozygous 257:erythrocytes 254: 249:hemoglobin A 233: 231: 211: 204: 199:target cells 183:blood smears 179:Target cells 168: 135:heterozygous 132: 120: 109:Harvey Itano 106: 101: 89: 83: 63: 60:Hemoglobin C 59: 58: 33:Hemoglobin C 2553:Hemoglobins 2456:Neuroglobin 2382:Other human 2095:HbE Gower 2 2081:HbE Gower 1 1802:MedlinePlus 1668:: 409–421. 1330:: 157–178. 562:(26): e24. 399:West Africa 273:homozygotes 2547:Categories 2503:Cytochrome 2461:Cytoglobin 2241:pathology: 2064:stages of 1979:Beta locus 1887:Hemoglobin 1778:DiseasesDB 676:The Lancet 627:2015-03-03 603:2015-03-03 486:2020-10-26 473:StatPearls 445:References 393:, and the 347:Folic acid 333:Prevention 259:causing a 171:microcytic 155:gallstones 139:homozygous 119:caused by 68:hemoglobin 44:Hematology 2531:see also 2444:Myoglobin 2347:Compounds 2218:HbF/Fetal 2148:HbF/Fetal 2074:Embryonic 2055:Tetramers 1813:eMedicine 1156:0025-1496 1142:: 34–36. 656:eMedicine 498:CC BY 4.0 395:Caribbean 353:Prognosis 341:Treatment 313:Diagnosis 294:P. vivax, 159:hemolysis 147:hemolytic 70:in which 39:Specialty 2403:Nonhuman 1895:Subunits 1861:Proteins 1682:13716852 1604:13187564 1565:13275117 1493:13192193 1444:14886398 1403:14808148 1305:13362221 1264:44763460 1256:20955412 1215:31674765 1207:15395398 1121:12480691 1085:24734397 1012:22634344 955:15973412 905:22445352 848:11713529 796:20231425 755:26019283 706:22445352 576:15215497 538:12818227 500:license. 481:32644469 240:β-globin 228:Genetics 76:β-globin 1878:Globins 1772:D006445 1643:2793520 1484:1023791 1394:1063254 1371:Bibcode 1344:4912473 1296:1716688 1187:Bibcode 1179:Science 1164:2793520 1077:2672962 1057:Bibcode 1003:3396718 963:4412263 933:Bibcode 896:3404513 856:4360808 828:Bibcode 746:4633765 697:3404513 409:History 403:malaria 374:Yorubas 321:(CBC), 2471:plant: 2436:human: 1930:pseudo 1807:000572 1715:(MeSH) 1705:(MeSH) 1680:  1641:  1629:: 34. 1602:  1563:  1491:  1481:  1442:  1401:  1391:  1342:  1303:  1293:  1262:  1254:  1213:  1205:  1162:  1154:  1119:  1083:  1075:  1010:  1000:  961:  953:  925:Nature 903:  893:  854:  846:  820:Nature 794:  753:  743:  704:  694:  574:  536:  479:  387:Greece 359:benign 325:, and 307:PfEMP1 269:anemia 143:anemia 94:anemia 80:lysine 46:  2496:Other 2428:Other 2259:Barts 2180:Adult 2141:Fetal 1783:29693 1761:282.7 1746:D58.2 1639:JSTOR 1512:Blood 1422:Blood 1260:S2CID 1211:S2CID 1160:JSTOR 1101:Blood 1081:S2CID 959:S2CID 852:S2CID 774:Blood 383:Italy 245:trait 2039:HBE1 2027:HBG2 2022:HBG1 1954:HBQ1 1925:HBA2 1920:HBA1 1865:heme 1767:MeSH 1756:9-CM 1678:PMID 1600:PMID 1561:PMID 1489:PMID 1440:PMID 1399:PMID 1340:PMID 1301:PMID 1252:PMID 1203:PMID 1152:ISSN 1117:PMID 1073:PMID 1008:PMID 951:PMID 901:PMID 844:PMID 792:PMID 751:PMID 702:PMID 572:PMID 534:PMID 477:PMID 421:and 220:and 111:and 2325:HbO 2311:HbE 2297:HbC 2283:HbS 2269:HbD 2249:HbH 2201:HbA 2187:HbA 2162:HbA 2010:HBD 1998:HBB 1981:on 1966:HBM 1942:HBZ 1752:ICD 1737:ICD 1670:doi 1631:doi 1623:Man 1592:doi 1553:doi 1520:doi 1479:PMC 1471:doi 1430:doi 1389:PMC 1379:doi 1332:doi 1291:PMC 1242:doi 1238:151 1195:doi 1183:110 1144:doi 1136:Man 1109:doi 1105:101 1065:doi 1053:565 998:PMC 990:doi 941:doi 929:435 891:PMC 883:doi 836:doi 824:414 782:doi 778:115 741:PMC 733:doi 729:212 692:PMC 684:doi 654:at 564:doi 560:350 526:doi 235:HBB 218:HbD 214:HbO 85:HBB 64:HbC 2549:: 2327:(α 2313:(α 2299:(α 2285:(α 2271:(α 2261:(γ 2251:(β 2220:(α 2206:(α 2189:(α 2164:(α 2150:(α 2125:(ζ 2111:(ζ 2097:(α 2083:(ζ 1983:11 1905:16 1816:: 1805:: 1781:: 1770:: 1759:: 1744:: 1741:10 1676:. 1666:42 1664:. 1660:. 1637:. 1627:56 1625:. 1621:. 1598:. 1588:48 1586:. 1582:. 1559:. 1549:91 1547:. 1543:. 1514:. 1510:. 1487:. 1477:. 1465:. 1461:. 1438:. 1424:. 1420:. 1397:. 1387:. 1377:. 1367:36 1365:. 1361:. 1338:. 1328:21 1326:. 1322:. 1299:. 1285:. 1281:. 1258:. 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Index

Hemoglobin SC disease
Specialty
Hematology
Edit this on Wikidata
hemoglobin
glutamic acid
β-globin
lysine
HBB
anemia
sickle cell anemia
Harvey Itano
James V. Neel
naturally resistant to malaria
Plasmodium falciparum
heterozygous
homozygous
anemia
hemolytic
enlarged spleen
gallstones
hemolysis
microcytic
mean corpuscular hemoglobin concentration
Target cells
blood smears

target cells
sickle cell disease
HbO

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