Showing papers by "Arno G. Motulsky published in 1966"

Predominance of hemoglobin Gower 1 in early human embryonic development.

Abstract: Hemoglobin Gower 1, the structure of which is thought to be epsilon(4), is the predominant hemoglobin in early human embryonic life. This finding suggests that the production of (epsilon)-chains initially exceeds that of other known (alpha, beta, gamma, and delta) chains.

Hemoglobin tacoma--a beta-chain variant associated with increased hb A2.

Abstract: A hitherto unknown inherited hemoglobin variant, Hb Tacoma, was discovered in three healthy members of a family of European extraction. Hybridization experiments with canine hemoglobin indicated a structural abnormality in the β-chain. The variant was therefore designated as Hb α2 β2 Tacoma. Separation of Hb Tacoma from Hb A could only be clearly achieved by starch grain electrophoresis in TEB buffer at pH 8.6–9.0, where Hb Tacoma moved more rapidly towards the anode than Hb A; gradient column chromatography on DEAE cellulose and CM-Sephadex achieved partial separation. The proportion of abnormal hemoglobin in the heterozygote amounted to 43 per cent of the total hemoglobin. Hb Tacoma was less heat resistant and became more rapidly denatured in 8 M urea solution than Hb A, Hb C, or Hb S. On thin-layer starch gel electrophoresis in TCB buffer at pH 8.6 Hb Tacoma was associated with an electrophoretically slow, benzidine-positive component, Hb Tacoma-slow. The exact biochemical nature of this minor component could not be determined, although a polymeric product of Hb Tacoma is suspected. Heterozygosity for Hb Tacoma is associated with a raised Hb A2 level without evidence of β-thalassemia.

Clinical Implications of Glucose-6-Phosphate Dehydrogenase Deficiency

Abstract: Excerpt The clinical significance of the deficiency of red-cell glucose-6-phosphate dehydrogenase (G-6-PD) was realized when this enzymatic abnormality was found to be the basic lesion responsible ...