Hemoglobin Hamilton or alpha 2 beta 2 11(A8)Val leads to Ile: a silent beta-chain variant detected by Triton X-100 acid-urea polyacrylamide gel electrophoresis
- PMID:6695908
- DOI: 10.1002/ajh.2830160106
Hemoglobin Hamilton or alpha 2 beta 2 11(A8)Val leads to Ile: a silent beta-chain variant detected by Triton X-100 acid-urea polyacrylamide gel electrophoresis
Abstract
A silent beta-chain hemoglobin variant, not detectable by starch gel or agar gel electrophoresis was found using Triton X-100 acid-urea polyacrylamide gel electrophoresis. The abnormal beta-X chain had a more anodic electrophoretic mobility; 38% of the total beta chain was of the abnormal type. Structural analysis using high-performance liquid chromatography and microsequencing procedures indicated a valine to isoleucine substitution at position beta 11(A8). This anomaly did not change the functional properties of the hemoglobin molecule. A mild reticulocytosis was observed in the propositus.
Similar articles
- Hb O-Tibesti [beta121(GH4)Glu-->Lys; beta11(A8)Val-->Ile], a hemoglobin variant carrying in the same beta chain the substitutions of Hb O-Arab and Hb Hamilton, found in combination with Hb S [beta6(A3)Glu-->Val].Préhu C, Riou J, Sartelet I, Promé D, Claparols C, Denier M, Motte J, Galactéros F, Wajcman H.Préhu C, et al.Hemoglobin. 2002 Feb;26(1):13-20. doi: 10.1081/hem-120002936.Hemoglobin. 2002.PMID:11939508
- Hemoglobin Syracuse (alpha2beta2-143(H21)His leads to Pro), a new high-affinity variant detected by special electrophoretic methods. Observations on the auto-oxidation of normal and variant hemoglobins.Jensen M, Oski FA, Nathan DG, Bunn HF.Jensen M, et al.J Clin Invest. 1975 Mar;55(3):469-77. doi: 10.1172/JCI107953.J Clin Invest. 1975.PMID:234980Free PMC article.
- Chemical evidence for the separation of G gamma and A gamma chains by polyacrylamide gel electrophoresis in urea and triton X-100.Alter BP, Goff SC, Klonowski TJ, Garrick MD.Alter BP, et al.Prep Biochem. 1980;10(5):633-44. doi: 10.1080/00327488008061758.Prep Biochem. 1980.PMID:6160570
- Two new cases of Hb Denmark Hill [alpha 95(G2)Pro----Ala] discovered in south-west France: electrophoretical and chromatographical considerations.Vasseur C, Francina A, Wajcman H, Galactéros F.Vasseur C, et al.Hemoglobin. 1991;15(1-2):103-8. doi: 10.3109/03630269109072489.Hemoglobin. 1991.PMID:1917529No abstract available.
- Hemoglobin Constant Spring, and unusual alpha-chain variant involved in the etiology of hemoglobin H disease.Clegg JB, Weatherall DJ.Clegg JB, et al.Ann N Y Acad Sci. 1974;232(0):168-78. doi: 10.1111/j.1749-6632.1974.tb20582.x.Ann N Y Acad Sci. 1974.PMID:4606609Review.No abstract available.
Cited by
- DNA polymorphisms in North Sardinian newborns and their linkage with abnormal gamma globin gene arrangements and with beta (0) -thalassemia.Hattori Y, Kutlar F, Chen SS, Huisman TH, Demuro P, Formato M, Manca L, Masala B.Hattori Y, et al.Biochem Genet. 1986 Oct;24(9-10):669-81. doi: 10.1007/BF00499001.Biochem Genet. 1986.PMID:3778425
Publication types
MeSH terms
Substances
Related information
Grants and funding
LinkOut - more resources
Full Text Sources