Document Detail

Biogenesis of erythrocyte membrane skeleton in health and disease.
MedLine Citation:
PMID:  8318923     Owner:  NLM     Status:  MEDLINE    
To study the biogenesis of red cell membrane skeleton at various stages of erythroid differentiation, we have chosen the following model systems: a) Rauscher erythroleukemia cell line representing the early stages of differentiation, b) Friend erythroleukemia cells, and c) in vitro cultured human erythroblasts. The latter two systems represent terminally differentiated erythroblasts. Using these model systems, we have shown asynchronous synthesis of membrane proteins during erythroid differentiation. At the early stages of erythroid development, the synthesis of spectrin, ankyrin and band 4.1 proteins is initiated before that of the band 3 protein. Following erythroid induction with erythropoietin and dimethylsulfoxide (DMSO), there is a dramatic increase in the synthesis of the band 3 protein without noticeable changes in the synthesis of other membrane proteins. This increase in band 3 synthesis is accompanied by increased stability and recruitment of the skeletal proteins into the membrane skeleton, leading to increased steady state levels. The progressive increase in band 3 synthesis continues during terminal maturation of erythroblasts. This is accompanied by increased stability and assembly of spectrin and ankyrin on the membrane, despite their reduced synthesis. These results point to a key role for the band 3 protein in anchoring and stabilizing these proteins into the permanent skeletal network. Finally, to detect defects of skeletal biosynthesis, we have extended these studies to a patient with severe hereditary spherocytosis characterized by a combined deficiency of spectrin and ankyrin. We have shown that this combined deficiency is a consequence of reduced ankyrin synthesis and mRNA content representing a thalassemia-like membrane protein mutation.
M Hanspal; J T Prchal; J Palek
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Stem cells (Dayton, Ohio)     Volume:  11 Suppl 1     ISSN:  1066-5099     ISO Abbreviation:  Stem Cells     Publication Date:  1993 May 
Date Detail:
Created Date:  1993-08-05     Completed Date:  1993-08-05     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9304532     Medline TA:  Stem Cells     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  8-12     Citation Subset:  IM    
Department of Biomedical Research, St. Elizabeth's Hospital of Boston, Boston, Massachusetts 02135.
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MeSH Terms
Ankyrins / biosynthesis
Cell Differentiation
Cells, Cultured
Erythrocyte Membrane / metabolism*,  ultrastructure
Leukemia, Erythroblastic, Acute
Microscopy, Electron
Spectrin / biosynthesis
Tumor Cells, Cultured
Reg. No./Substance:
0/Ankyrins; 12634-43-4/Spectrin

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