Abstract
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.
Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
