Oxidative instability of hemoglobin E (β26 Glu→Lys) is increased in the presence of free α subunits and reversed by α-hemoglobin stabilizing protein (AHSP): Relevance to HbE/β-thalassemia

Oxidative instability of hemoglobin E (β26 Glu→Lys) is increased in the presence of free α subunits and reversed by α-hemoglobin stabilizing protein (AHSP): Relevance to HbE/β-thalassemia

When adding peroxide (H2O2), β subunits of hemoglobin (Hb) bear the burden of oxidative changes due in part to the direct oxidation of its Cys93. The presence of unpaired α subunits within red cells and/or co-inheritance of another β subunit mutant, HbE (β26 Glu→Lys) have been implicated in the path...

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Bibliographic Details
Main Authors: Michael Brad Strader, Tigist Kassa, Fantao Meng, Francine B. Wood, Rhoda Elison Hirsch, Joel M. Friedman, Abdu I. Alayash
Format: Article
Language:English
Published: Elsevier 2016-08-01
Series:Redox Biology
Subjects:
Hemoglobin E
Thalassemia
Oxidation
Alpha-hemoglobin stabilizing protein
Online Access:http://www.sciencedirect.com/science/article/pii/S2213231716300234

Internet

http://www.sciencedirect.com/science/article/pii/S2213231716300234

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