Role of alpha-hemoglobin molecular chaperone in the hemoglobin formation and clinical expression of some hemoglobinopathies

Transfusion clinique et biologique : journal de la Société française de transfusion sanguine
Corinne Vasseur, Véronique Baudin-Creuza

Abstract

Alpha-hemoglobin stabilizing protein (AHSP), described as a chaperone of alpha-hemoglobin (α-Hb), is synthesized at a high concentration in the erythroid precursors. AHSP specifically recognizes the G and H helices of α-Hb and forms a stable complex with free α-Hb until its association with the partner β-subunits. Unlike the free β-Hb which are soluble and form homologous tetramers, freshly synthesized α-Hb chains are highly unstable molecular species which precipitate and generate reactive oxygen species within the erythrocyte precursors of the bone marrow leading to apoptosis and ineffective erythropoiesis. AHSP protects the free α-Hb chains in maintaining it in the soluble state. In this review, we report data from the literature and our laboratory concerning the key role of AHSP in the biosynthesis of Hb and its possible involvement in some disorders of the red blood cell as well as the hemoglobinopathies and we discuss its use as a prognostic tool in thalassemia syndromes.

References

Jun 18, 2002·Nature·Anthony J KihmMitchell J Weiss
Apr 23, 2004·Experimental Hematology·Camila Oresco dos SantosFernando Ferreira Costa
Jun 29, 2004·The Journal of Biological Chemistry·Véronique Baudin-CreuzaMichael C Marden
Jul 20, 2006·Nature Chemical Biology·Tracey A Rouault
Dec 26, 2008·Blood·Mitchell J Weiss, Camila O dos Santos
Jun 2, 2009·Clinical Biochemistry·Corinne VasseurVéronique Baudin-Creuza
Jan 26, 2011·American Journal of Hematology·Corinne VasseurVéronique Baudin-Creuza
Sep 18, 2012·The American Journal of Surgical Pathology·Philipp W RaessJohn Kim Choi
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Citations

Jun 3, 2017·Biomolecular Concepts·Giovanna De SimoneFabio Polticelli
Jul 26, 2016·Experimental and Therapeutic Medicine·Xinxian ZhangQiancheng Li
Sep 22, 2016·Antioxidants & Redox Signaling·Rhoda Elison HirschJoel M Friedman

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