Fate of alpha-hemoglobin chains and erythrocyte defects in beta-thalassemia.

Abstract:

:The fate of alpha-hemoglobin chains and the cause of membrane protein defects in thalassemic erythrocytes have been studied in: (1) human beta-thalassemia syndromes, (2) mouse beta-thalassemia, and (3) normal human erythrocytes loaded with purified alpha-hemoglobin chains. The similarity and differences observed in these three systems underline the importance of insoluble alpha chains and the direct relationship between the amount of these chains and the membrane protein defects. Indeed, in addition to the alpha/non-alpha ratio of globin chain synthesis, the proteolysis and instability of alpha chains are major factors in modulating the cellular defects.

journal_name

Ann N Y Acad Sci

authors

Rouyer-Fessard P,Scott MD,Leroy-Viard K,Garel MC,Bachir D,Galacteros F,Beuzard Y

doi

10.1111/j.1749-6632.1990.tb24296.x

subject

Has Abstract

pub_date

1990-01-01 00:00:00

pages

106-17

eissn

0077-8923

issn

1749-6632

journal_volume

612

pub_type

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