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The Interaction of α‐Thalassaemia and Haemoglobin G Philadelphia
Author(s) -
Rieder R. F.,
Woodbury D. H.,
Rucknagel D. L.
Publication year - 1976
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.1365-2141.1976.tb00918.x
Subject(s) - gene , genetics , biology , medicine , microbiology and biotechnology
An American Negro woman was found to have HbH disease in association with HbG Philadelphia (α68‐asnlys). Starch gel electrophoresis failed to reveal the presence of any HbA or HbA 2 and studies of globin chain synthesis indicated absence of α A production. The α G /β synthesis ratio was 0.63. The woman's son and her two half‐sibs had α‐thalassaemia trait with no HbH and α/β synthesis ratios of 0.84, 0.84 and 0.76. The data indicate that there is no functioning α A gene linked to the α G gene. The absence of α A synthesis by the propositus also indicates that the α‐thalassaemia gene trans to the α G gene completely suppresses α chain production, the first evidence for such a gene in Negroes.