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Genetic modulation of sickle cell anemia

Journal Article · · Proceedings of the Society for Experimental Biology and Medicine
 [1]
  1. Univ. of Mississippi School of Medicine, Jackson, MS (United States)
Sickle cell anemia, a common disorder associated with reduced life span of the red blood cell and vasoocclusive events, is caused by a mutation in the {Beta}-hemoglobin gene. Yet, despite this genetic homogeneity, the phenotype of the disease is heterogeneous. This suggests the modulating influence of associated inherited traits. Some of these may influence the accumulation of fetal hemoglobin, a hemoglobin type that interferes with the polymerization of sickle hemoglobin. Another inherited trait determines the accumulation of {alpha}-globin chains. This review focuses on potential genetic regulators of the phenotype of sickle cell anemia. 125 refs., 6 figs., 3 tabs.
Sponsoring Organization:
USDOE
OSTI ID:
209967
Journal Information:
Proceedings of the Society for Experimental Biology and Medicine, Journal Name: Proceedings of the Society for Experimental Biology and Medicine Journal Issue: 1 Vol. 209; ISSN PSEBAA; ISSN 0037-9727
Country of Publication:
United States
Language:
English

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