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.
251:
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.
1098:
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".
918:
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).
296:
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.
242:
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
291:
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
380:
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
719:
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).
768:
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).
397:
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
88:
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
437:
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,
301:
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
814:
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.
1507:
425:
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.
161:
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
169:
The red blood cells of people with hemoglobin C disease are usually abnormally small (
2546:
2519:
2478:
2393:
2200:
1982:
1965:
1904:
1673:
1595:
1246:
1229:
438:
426:
418:
390:
337:
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:
1771:
1198:
993:
786:
769:
554:
Fairhurst, Rick M.; Casella, James F. (2004). "Homozygous
Hemoglobin C Disease".
2455:
1868:
1801:
1030:
398:
198:
182:
1782:
1363:
Proceedings of the
National Academy of Sciences of the United States of America
1177:
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).
670:
Taylor, Steve M.; Parobek, Christian M.; Fairhurst, Rick M. (2012).
1929:
1584:
Transactions of the Royal Society of Tropical Medicine and Hygiene
382:
192:
976:
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
212:
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:
2178:
2163:
2160:
2149:
2146:
2145:
2143:
2139:
2124:
2121:
2110:
2107:
2096:
2093:
2082:
2079:
2078:
2076:
2072:
2069:
2067:
2061:
2058:
2056:
2052:
2040:
2037:
2036:
2035:
2032:
2028:
2025:
2023:
2020:
2019:
2018:
2015:
2011:
2008:
2007:
2006:
2003:
1999:
1996:
1995:
1994:
1991:
1990:
1988:
1986:
1984:
1980:
1975:
1967:
1964:
1963:
1962:
1959:
1955:
1952:
1951:
1950:
1947:
1943:
1940:
1939:
1938:
1935:
1931:
1928:
1926:
1923:
1921:
1918:
1917:
1916:
1913:
1912:
1910:
1908:
1906:
1900:
1897:
1893:
1890:
1888:
1884:
1881:
1879:
1875:
1870:
1866:
1863:that contain
1862:
1855:
1850:
1848:
1843:
1841:
1836:
1835:
1832:
1819:
1815:
1814:
1810:
1808:
1804:
1803:
1799:
1798:
1795:
1791:
1784:
1780:
1779:
1775:
1773:
1769:
1768:
1764:
1762:
1758:
1757:
1753:
1749:
1747:
1743:
1742:
1738:
1734:
1733:
1730:
1725:
1721:
1714:
1710:
1707:
1704:
1700:
1697:
1696:
1692:
1683:
1679:
1675:
1671:
1667:
1663:
1659:
1652:
1649:
1644:
1640:
1636:
1632:
1628:
1624:
1620:
1613:
1610:
1605:
1601:
1597:
1593:
1589:
1585:
1581:
1574:
1571:
1566:
1562:
1558:
1554:
1550:
1546:
1542:
1535:
1532:
1526:
1521:
1517:
1513:
1509:
1502:
1499:
1494:
1490:
1485:
1480:
1476:
1472:
1468:
1464:
1460:
1453:
1450:
1445:
1441:
1436:
1431:
1427:
1423:
1419:
1412:
1409:
1404:
1400:
1395:
1390:
1385:
1380:
1376:
1372:
1368:
1364:
1360:
1353:
1350:
1345:
1341:
1337:
1333:
1329:
1325:
1321:
1314:
1311:
1306:
1302:
1297:
1292:
1288:
1284:
1280:
1273:
1270:
1265:
1261:
1257:
1253:
1248:
1243:
1239:
1235:
1231:
1224:
1221:
1216:
1212:
1208:
1204:
1200:
1196:
1192:
1188:
1184:
1180:
1173:
1170:
1165:
1161:
1157:
1153:
1149:
1145:
1141:
1137:
1130:
1127:
1122:
1118:
1114:
1110:
1106:
1102:
1094:
1091:
1086:
1082:
1078:
1074:
1070:
1066:
1062:
1058:
1055:(1): 126–36.
1054:
1050:
1043:
1040:
1037:
1033:
1032:
1026:
1024:
1022:
1018:
1013:
1009:
1004:
999:
995:
991:
987:
983:
979:
972:
969:
964:
960:
956:
952:
947:
942:
938:
934:
930:
926:
922:
914:
911:
906:
902:
897:
892:
888:
884:
880:
876:
872:
865:
862:
857:
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:.
1250:.
1236:.
1232:.
1209:.
1201:.
1193:.
1181:.
1158:.
1150:.
1140:56
1138:.
1115:.
1103:.
1079:.
1071:.
1063:.
1051:.
1034::
1020:^
1006:.
996:.
986:14
984:.
980:.
957:.
949:.
939:.
927:.
923:.
899:.
889:.
879:12
877:.
873:.
850:.
842:.
834:.
822:.
818:.
804:^
790:.
776:.
772:.
749:.
739:.
727:.
723:.
700:.
690:.
680:12
678:.
674:.
662:^
636:^
612:^
584:^
570:.
558:.
546:^
532:.
522:17
520:.
506:^
471:,
453:^
417:,
389:,
385:,
329:.
224:.
216:,
2366:/
2335:)
2333:2
2331:β
2329:2
2321:)
2319:2
2317:β
2315:2
2307:)
2305:2
2303:β
2301:2
2293:)
2291:2
2289:β
2287:2
2279:)
2277:2
2275:β
2273:2
2265:)
2263:4
2255:)
2253:4
2228:)
2226:2
2224:γ
2222:2
2214:)
2212:2
2210:δ
2208:2
2203:2
2197:)
2195:2
2193:β
2191:2
2172:)
2170:2
2168:β
2166:2
2158:)
2156:2
2154:γ
2152:2
2133:)
2131:2
2129:β
2127:2
2119:)
2117:2
2115:γ
2113:2
2105:)
2103:2
2101:ε
2099:2
2091:)
2089:2
2087:ε
2085:2
2034:ε
2017:γ
2005:δ
1993:β
1985::
1961:μ
1949:θ
1937:ζ
1915:α
1907::
1871:)
1867:(
1853:e
1846:t
1839:v
1754:-
1739:-
1729:D
1684:.
1672::
1645:.
1633::
1606:.
1594::
1567:.
1555::
1528:.
1522::
1516:8
1495:.
1473::
1467:7
1446:.
1432::
1426:6
1405:.
1381::
1373::
1346:.
1334::
1307:.
1287:8
1266:.
1244::
1217:.
1197::
1189::
1166:.
1146::
1123:.
1111::
1087:.
1067::
1059::
1014:.
992::
965:.
943::
935::
907:.
885::
858:.
838::
830::
798:.
784::
757:.
735::
708:.
686::
630:.
606:.
578:.
566::
540:.
528::
20:)
Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.