AHSP: a novel hemoglobin helper
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AHSP: a novel hemoglobin helper.
Recently, the small protein alpha hemoglobin-stabilizing protein (AHSP) was identified and found to specifically bind alpha-globin, stabilize its structure, and limit the toxic effects of excess alpha-globin, which are manifest in the inherited blood disorder beta thalassemia. In this issue of the JCI, Yu, Weiss, and colleagues show that AHSP is also critical to the formation and stabilization ...
متن کاملMolecular Mechanism of AHSP-Mediated Stabilization of α-Hemoglobin
ized by high-level production of hemoglobin A (HbA), which is composed of two ␣ and two  subunits, each bound to a heme moiety and produced in a concerted * the bound heme; oxygen binds cooperatively and re-* versibly to ferrous iron from the opposite side of the 1 Department of Molecular Biology heme plane. The elaborate oxygen binding mechanism Lewis Thomas Laboratory of HbA allows the conce...
متن کاملEvaluation of alpha hemoglobin stabilizing protein (AHSP) as a genetic modifier in patients with beta thalassemia.
Although beta thalassemia is considered to be a classic monogenic disease, it is clear that there is considerable clinical variability between patients who inherit identical beta globin gene mutations, suggesting that there may be a variety of genetic determinants influencing different clinical phenotypes. It has been suggested that variations in the structure or amounts of a highly expressed r...
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Alpha-Hemoglobin Stabilizing Protein (AHSP) binds to α-hemoglobin (α-Hb) or α-globin and maintains it in a soluble state until its association with the β-Hb chain partner to form Hb tetramers. AHSP specifically recognizes the G and H helices of α-Hb. To investigate the degree of interaction of the various regions of the α-globin H helix with AHSP, this interface was studied by stepwise eliminat...
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ژورنال
عنوان ژورنال: Journal of Clinical Investigation
سال: 2007
ISSN: 0021-9738
DOI: 10.1172/jci32362