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| {{STRUCTURE_1y01| PDB=1y01 | SCENE= }} | | {{STRUCTURE_1y01| PDB=1y01 | SCENE= }} |
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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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| ==Overview==
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| 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.
| | The line below this paragraph, {{ABSTRACT_PUBMED_15550245}}, adds the Publication Abstract to the page |
| | (as it appears on PubMed at http://www.pubmed.gov), where 15550245 is the PubMed ID number. |
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| | {{ABSTRACT_PUBMED_15550245}} |
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| ==Disease== | | ==Disease== |
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| [[Category: Recognition]] | | [[Category: Recognition]] |
| [[Category: Stabilization of alpha hemoglobin]] | | [[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 Sun Jul 27 23:24:47 2008'' |
