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. 1979 Jul;76(7):3420–3424. doi: 10.1073/pnas.76.7.3420

Human T gamma globin chain is a variant of A gamma chain (A gamma Sardinia).

G Saglio, G Ricco, U Mazza, C Camaschella, P G Pich, A M Gianni, E Gianazza, P G Righetti, B Giglioni, P Comi, M Gusmeroli, S Ottolenghi
PMCID: PMC383837  PMID: 291015

Abstract

Isoelectric focusing, cellulose acetate electrophoresis, and carboxymethylcellulose chromatography in the presence of Nonidet P-40 allow the separation of pure gamma chains into two fractions. Amino acid analysis of their cyanogen bromide fragment 3 (gamma CB3) identifies these fractions as the separated G gamma (Gly-136) and A gamma (Ala-136) globin chains. Fingerprint and amino acid analyses of the gamma Tp9 tryptic peptide from the purified A gamma and G gamma fractions from two different patients demonstrate that the commonly occurring gamma Sardinia variant (gamma 75 isoleucine leads to threonine), also known as T gamma chain, has alanine in position 136. From this analysis we suggest that the T gamma gene is an allele of the A gamma locus (A gamma Sardinia) rather than a third gamma locus.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. BAGLIONI C. An improved method for the fingerprinting of human hemoglobin. Biochim Biophys Acta. 1961 Apr 1;48:392–396. doi: 10.1016/0006-3002(61)90490-5. [DOI] [PubMed] [Google Scholar]
  2. Beale D. A partial amino acid sequence for sheep haemoblogin A. Biochem J. 1967 Apr;103(1):129–140. doi: 10.1042/bj1030129. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Clegg J. B., Naughton M. A., Weatherball D. J. Abnormal human haemoglobins. Separation and characterization of the alpha and beta chains by chromatography, and the determination of two new variants, hb Chesapeak and hb J (Bangkok). J Mol Biol. 1966 Aug;19(1):91–108. doi: 10.1016/s0022-2836(66)80052-9. [DOI] [PubMed] [Google Scholar]
  4. Comi P., Giglioni B., Ottolenghi S., Gianni A. M., Ricco G., Mazza U., Saglio G., Camaschella C., Pich P. G., Gianazza E. G gamma and A gamma globin chains separation and quantitation by isoelectric focusing. Biochem Biophys Res Commun. 1979 Mar 15;87(1):1–8. doi: 10.1016/0006-291x(79)91639-5. [DOI] [PubMed] [Google Scholar]
  5. Grifoni V., Kamuzora H., Lehmann H., Charlesworth D. A new Hb variant: Hb F Sardinia gamma75(E19) isoleucine leads to threonine found in a family with Hb G Philadelphia, beta-chain deficiency and a Lepore-like haemoglobin indistinguishable from Hb A2. Acta Haematol. 1975;53(6):347–355. doi: 10.1159/000208204. [DOI] [PubMed] [Google Scholar]
  6. Huisman T. H., Harris H., Gravely M. The chemical heterogeneity of the fetal hemoglobin in normal newborn infants and in adults. Mol Cell Biochem. 1977 Aug 19;17(1):45–55. doi: 10.1007/BF01732554. [DOI] [PubMed] [Google Scholar]
  7. Huisman T. H., Schroeder W. A., Efremov G. D., Duma H., Mladenovski B., Hyman C. B., Rachmilewitz E. A., Bouver N., Miller A., Brodie A. The present status of the heterogeneity of fetal hemoglobin in beta-thalassemia: an attempt to unify some observations in thalassemia and related conditions. Ann N Y Acad Sci. 1974;232(0):107–124. doi: 10.1111/j.1749-6632.1974.tb20576.x. [DOI] [PubMed] [Google Scholar]
  8. INGRAM V. M. Abnormal human haemoglobins. I. The comparison of normal human and sickle-cell haemoglobins by fingerprinting. Biochim Biophys Acta. 1958 Jun;28(3):539–545. doi: 10.1016/0006-3002(58)90516-x. [DOI] [PubMed] [Google Scholar]
  9. Kamuzora H., Ringelhann B., Konotey-Ahulu F. I., Lehmann H., Lorkin P. A. Futher investigations of the gamma-chaina in a ghanaian adult, homozygous for hereditary perisitence of fetal haemoglobin. Isolation of gamma CB-3 peptides and Ggamma:Agamma ratio determination in human Hb E. Acta Haematol. 1975;53(5):315–320. doi: 10.1159/000208198. [DOI] [PubMed] [Google Scholar]
  10. Old J., Clegg J. B., Weatherall D. J., Ottolenghi S., Comi P., Giglioni B., Mitchell J., Tolstoshev P., Williamson R. A direct estimate of the number of human gamma-globin genes. Cell. 1976 May;8(1):13–18. doi: 10.1016/0092-8674(76)90180-x. [DOI] [PubMed] [Google Scholar]
  11. Ricco G., Mazza U., Turi R. M., Pich P. G., Camaschella C., Saglio G., Bernini L. F. Significance of a new type of human fetal hemoglobin carrying a replacement isoleucine replaced by threonine at position 75 )E 19) of the gamma chain. Hum Genet. 1976 Jun 29;32(3):305–313. doi: 10.1007/BF00295821. [DOI] [PubMed] [Google Scholar]
  12. Righetti P. G., Gianazza E., Gianni A. M., Comi P., Giglioni B., Ottolenghi S., Secchi C., Rossi-Bernardi L. Human globin chain separation by isoelectric focusing. J Biochem Biophys Methods. 1979;1(1):45–57. doi: 10.1016/0165-022x(79)90045-9. [DOI] [PubMed] [Google Scholar]
  13. Schroeder W. A., Bannister W. H., Grech J. L., Brown A. K., Wrightstone R. N., Huisman T. H. Non-synchronized suppression of postnatal activity in non-allelic genes which synthesize the Ggamma chain in human foetal haemoglobin. Nat New Biol. 1973 Jul 18;244(133):89–90. doi: 10.1038/newbio244089a0. [DOI] [PubMed] [Google Scholar]
  14. Schroeder W. A., Huisman T. H., Shelton J. R., Shelton J. B., Kleihauer E. F., Dozy A. M., Robberson B. Evidence for multiple structural genes for the gamma chain of human fetal hemoglobin. Proc Natl Acad Sci U S A. 1968 Jun;60(2):537–544. doi: 10.1073/pnas.60.2.537. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Schroeder W. A., Shelton J. R., Shelton J. B., Apell G., Huisman T. H., Bouver N. G. World-wide occurrence of nonallelic genes for the -chain of human foetal haemoglobin in newborns. Nat New Biol. 1972 Dec 27;240(104):273–274. doi: 10.1038/newbio240273a0. [DOI] [PubMed] [Google Scholar]
  16. Sick K., Beale D., Irvine D., Lehmann H., Goodall P. T., MacDougall S. Haemoglobin G Copenhagen and haemoglobin J Cambridge. Two new beta-chain variants of haemoglobin A. Biochim Biophys Acta. 1967 Jun 27;140(2):231–242. doi: 10.1016/0005-2795(67)90463-1. [DOI] [PubMed] [Google Scholar]
  17. Ui N. Conformational studies on proteins by isoelectric focusing. Ann N Y Acad Sci. 1973 Jun 15;209:198–209. doi: 10.1111/j.1749-6632.1973.tb47529.x. [DOI] [PubMed] [Google Scholar]

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