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The Synthesis of Globin Peptide Chains in Sickle‐Cell Disease
Author(s) -
Sarup B. M.,
White J. M.
Publication year - 1974
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1974.tb06782.x
Subject(s) - globin , heterozygote advantage , cell , chemistry , microbiology and biotechnology , biology , hemoglobin , medicine , biochemistry , gene , allele
S ummary . The relative rates of synthesis of the globin chains (α, β s and γ) have been measured in vitro in the reticulocytes of eight patients homozygous for Hb S. The results indicate that the synthesis of the β s chain and its association with α chains are normal in the majority of homozygous patients. In two patients an excess synthesis of α chains was detected. The cause for this has not been determined, but it appears to be a transient phenomenon. The explanation is discussed for the normal synthesis of the β s globin in home zygotes and the fact that in sickle heterozygotes the specific activities of α‐ and β‐chains have usually been unity, i.e. that they are being produced at the same rate as they appear in the peripheral blood is discussed.