1z8u

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[[Image:1z8u.gif|left|200px]]<br /><applet load="1z8u" size="350" color="white" frame="true" align="right" spinBox="true"
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[[Image:1z8u.gif|left|200px]]
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caption="1z8u, resolution 2.4&Aring;" />
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'''Crystal structure of oxidized alpha hemoglobin bound to AHSP'''<br />
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{{Structure
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|PDB= 1z8u |SIZE=350|CAPTION= <scene name='initialview01'>1z8u</scene>, resolution 2.4&Aring;
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|SITE=
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|LIGAND= <scene name='pdbligand=HEM:PROTOPORPHYRIN IX CONTAINING FE'>HEM</scene>
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|ACTIVITY=
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|GENE= AHSP, EDRF, ERAF ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens]), HBA1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
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}}
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'''Crystal structure of oxidized alpha hemoglobin bound to AHSP'''
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==Overview==
==Overview==
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==About this Structure==
==About this Structure==
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1Z8U is a [http://en.wikipedia.org/wiki/Protein_complex Protein complex] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with <scene name='pdbligand=HEM:'>HEM</scene> as [http://en.wikipedia.org/wiki/ligand ligand]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1Z8U OCA].
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1Z8U is a [[Protein complex]] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1Z8U OCA].
==Reference==
==Reference==
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Structure of oxidized alpha-haemoglobin bound to AHSP reveals a protective mechanism for haem., Feng L, Zhou S, Gu L, Gell DA, Mackay JP, Weiss MJ, Gow AJ, Shi Y, Nature. 2005 Jun 2;435(7042):697-701. PMID:[http://ispc.weizmann.ac.il//pmbin/getpm?pmid=15931225 15931225]
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Structure of oxidized alpha-haemoglobin bound to AHSP reveals a protective mechanism for haem., Feng L, Zhou S, Gu L, Gell DA, Mackay JP, Weiss MJ, Gow AJ, Shi Y, Nature. 2005 Jun 2;435(7042):697-701. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/15931225 15931225]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Protein complex]]
[[Category: Protein complex]]
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[[Category: oxidation]]
[[Category: oxidation]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 16:13:15 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 15:32:33 2008''

Revision as of 13:32, 20 March 2008

Image:1z8u.gif


PDB ID 1z8u

Drag the structure with the mouse to rotate
, resolution 2.4Å
Ligands:
Gene: AHSP, EDRF, ERAF (Homo sapiens), HBA1 (Homo sapiens)
Coordinates: save as pdb, mmCIF, xml



Crystal structure of oxidized alpha hemoglobin bound to AHSP


Contents

Overview

The synthesis of haemoglobin A (HbA) is exquisitely coordinated during erythrocyte development to prevent damaging effects from individual alpha- and beta-subunits. The alpha-haemoglobin-stabilizing protein (AHSP) binds alpha-haemoglobin (alphaHb), inhibits the ability of alphaHb to generate reactive oxygen species and prevents its precipitation on exposure to oxidant stress. The structure of AHSP bound to ferrous alphaHb is thought to represent a transitional complex through which alphaHb is converted to a non-reactive, hexacoordinate ferric form. Here we report the crystal structure of this ferric alphaHb-AHSP complex at 2.4 A resolution. Our findings reveal a striking bis-histidyl configuration in which both the proximal and the distal histidines coordinate the haem iron atom. To attain this unusual conformation, segments of alphaHb undergo drastic structural rearrangements, including the repositioning of several alpha-helices. Moreover, conversion to the ferric bis-histidine configuration strongly and specifically inhibits redox chemistry catalysis and haem loss from alphaHb. The observed structural changes, which impair the chemical reactivity of haem iron, explain how AHSP stabilizes alphaHb and prevents its damaging effects in cells.

Disease

Known diseases associated with this structure: Erythremias, alpha- OMIM:[141800], Erythrocytosis OMIM:[141850], Heinz body anemia OMIM:[141850], Heinz body anemias, alpha- OMIM:[141800], Hemoglobin H disease OMIM:[141850], Hypochromic microcytic anemia OMIM:[141850], Methemoglobinemias, alpha- OMIM:[141800], Thalassemia, alpha- OMIM:[141850], Thalassemias, alpha- OMIM:[141800]

About this Structure

1Z8U is a Protein complex structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Structure of oxidized alpha-haemoglobin bound to AHSP reveals a protective mechanism for haem., Feng L, Zhou S, Gu L, Gell DA, Mackay JP, Weiss MJ, Gow AJ, Shi Y, Nature. 2005 Jun 2;435(7042):697-701. PMID:15931225

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