1y01
From Proteopedia
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'''Crystal structure of AHSP bound to Fe(II) alpha-hemoglobin''' | '''Crystal structure of AHSP bound to Fe(II) alpha-hemoglobin''' | ||
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[[Category: Weiss, M J.]] | [[Category: Weiss, M J.]] | ||
[[Category: Zhou, S.]] | [[Category: Zhou, S.]] | ||
| - | [[Category: | + | [[Category: Ahsp]] |
| - | [[Category: | + | [[Category: Alpha hemoglobin]] |
| - | [[Category: | + | [[Category: Hemoglobin oxidation and precipitation]] |
| - | [[Category: | + | [[Category: Recognition]] |
| - | [[Category: | + | [[Category: Stabilization of alpha hemoglobin]] |
| - | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 15:43:05 2008'' | |
| - | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on | + | |
Revision as of 12:43, 3 May 2008
Crystal structure of AHSP bound to Fe(II) alpha-hemoglobin
Contents |
Overview
Hemoglobin A (HbA), the oxygen delivery system in humans, comprises two alpha and two beta subunits. Free alpha-hemoglobin (alphaHb) is unstable, and its precipitation contributes to the pathophysiology of beta thalassemia. In erythrocytes, the alpha-hemoglobin stabilizing protein (AHSP) binds alphaHb and inhibits its precipitation. The crystal structure of AHSP bound to Fe(II)-alphaHb reveals that AHSP specifically recognizes the G and H helices of alphaHb through a hydrophobic interface that largely recapitulates the alpha1-beta1 interface of hemoglobin. The AHSP-alphaHb interactions are extensive but suboptimal, explaining why beta-hemoglobin can competitively displace AHSP to form HbA. Remarkably, the Fe(II)-heme group in AHSP bound alphaHb is coordinated by the distal but not the proximal histidine. Importantly, binding to AHSP facilitates the conversion of oxy-alphaHb to a deoxygenated, oxidized [Fe(III)], nonreactive form in which all six coordinate positions are occupied. These observations reveal the molecular mechanisms by which AHSP stabilizes free alphaHb.
Disease
Known disease associated with this structure: Erythremias, alpha- OMIM:[141800], Heinz body anemias, alpha- OMIM:[141800], Methemoglobinemias, alpha- OMIM:[141800], Thalassemias, alpha- OMIM:[141800]
About this Structure
1Y01 is a Protein complex structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Molecular mechanism of AHSP-mediated stabilization of alpha-hemoglobin., Feng L, Gell DA, Zhou S, Gu L, Kong Y, Li J, Hu M, Yan N, Lee C, Rich AM, Armstrong RS, Lay PA, Gow AJ, Weiss MJ, Mackay JP, Shi Y, Cell. 2004 Nov 24;119(5):629-40. PMID:15550245 Page seeded by OCA on Sat May 3 15:43:05 2008
