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Alpha-Hemoglobin-Stabilizing Protein : An Erythroid Molecular Chaperone
Joint Authors
Costa, Fernando Ferreira
Favero, Maria Emília
Source
Biochemistry Research International
Issue
Vol. 2011, Issue 2011 (31 Dec. 2011), pp.1-7, 7 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2011-03-24
Country of Publication
Egypt
No. of Pages
7
Main Subjects
Abstract EN
Alpha-hemoglobin-stabilizing protein (AHSP) is an erythroid-specific protein that acts as a molecular chaperone for the free α chains of hemoglobin.
Evidence strongly suggests that AHSP participates in hemoglobin synthesis and may act to neutralize the cytotoxic effects of excess free alpha-globin subunits that accumulate both in normal and beta-thalassemic erythroid precursor cells.
As such, AHSP seems to be essential for normal erythropoiesis, and impaired upregulation of AHSP may lead to premature erythroid cell death, resulting in ineffective erythropoiesis.
Reduced AHSP mRNA expression has been associated with clinical variability in some cases of β-thalassemia.
It has been shown that αHb variants may also impair AHSP-αHb interactions, leading to pathological conditions that resemble α-thalassemia syndromes.
The aim of this paper is to summarize current information concerning the structure and function of AHSP, focusing on its role in normal erythropoiesis and its relevance in health and disease.
American Psychological Association (APA)
Favero, Maria Emília& Costa, Fernando Ferreira. 2011. Alpha-Hemoglobin-Stabilizing Protein : An Erythroid Molecular Chaperone. Biochemistry Research International،Vol. 2011, no. 2011, pp.1-7.
https://search.emarefa.net/detail/BIM-466968
Modern Language Association (MLA)
Favero, Maria Emília& Costa, Fernando Ferreira. Alpha-Hemoglobin-Stabilizing Protein : An Erythroid Molecular Chaperone. Biochemistry Research International No. 2011 (2011), pp.1-7.
https://search.emarefa.net/detail/BIM-466968
American Medical Association (AMA)
Favero, Maria Emília& Costa, Fernando Ferreira. Alpha-Hemoglobin-Stabilizing Protein : An Erythroid Molecular Chaperone. Biochemistry Research International. 2011. Vol. 2011, no. 2011, pp.1-7.
https://search.emarefa.net/detail/BIM-466968
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-466968