2a1x

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{{STRUCTURE_2a1x| PDB=2a1x | SCENE= }}
{{STRUCTURE_2a1x| PDB=2a1x | SCENE= }}
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'''Human phytanoyl-coa 2-hydroxylase in complex with iron and 2-oxoglutarate'''
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===Human phytanoyl-coa 2-hydroxylase in complex with iron and 2-oxoglutarate===
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==Overview==
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Refsum disease (RD), a neurological syndrome characterized by adult onset retinitis pigmentosa, anosmia, sensory neuropathy, and phytanic acidaemia, is caused by elevated levels of phytanic acid. Many cases of RD are associated with mutations in phytanoyl-CoA 2-hydroxylase (PAHX), an Fe(II) and 2-oxoglutarate (2OG)-dependent oxygenase that catalyzes the initial alpha-oxidation step in the degradation of phytenic acid in peroxisomes. We describe the x-ray crystallographic structure of PAHX to 2.5 A resolution complexed with Fe(II) and 2OG and predict the molecular consequences of mutations causing RD. Like other 2OG oxygenases, PAHX possesses a double-stranded beta-helix core, which supports three iron binding ligands (His(175), Asp(177), and His(264)); the 2-oxoacid group of 2OG binds to the Fe(II) in a bidentate manner. The manner in which PAHX binds to Fe(II) and 2OG together with the presence of a cysteine residue (Cys(191)) 6.7 A from the Fe(II) and two further histidine residues (His(155) and His(281)) at its active site distinguishes it from that of the other human 2OG oxygenase for which structures are available, factor inhibiting hypoxia-inducible factor. Of the 15 PAHX residues observed to be mutated in RD patients, 11 cluster in two distinct groups around the Fe(II) (Pro(173), His(175), Gln(176), Asp(177), and His(220)) and 2OG binding sites (Trp(193), Glu(197), Ile(199), Gly(204), Asn(269), and Arg(275)). PAHX may be the first of a new subfamily of coenzyme A-binding 2OG oxygenases.
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{{ABSTRACT_PUBMED_16186124}}
==About this Structure==
==About this Structure==
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[[Category: Structural genomic]]
[[Category: Structural genomic]]
[[Category: Structural genomics consortium]]
[[Category: Structural genomics consortium]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 18:30:00 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Jul 28 12:45:40 2008''

Revision as of 09:45, 28 July 2008

Image:2a1x.png

Template:STRUCTURE 2a1x

Human phytanoyl-coa 2-hydroxylase in complex with iron and 2-oxoglutarate

Template:ABSTRACT PUBMED 16186124

About this Structure

2A1X is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease., McDonough MA, Kavanagh KL, Butler D, Searls T, Oppermann U, Schofield CJ, J Biol Chem. 2005 Dec 9;280(49):41101-10. Epub 2005 Sep 25. PMID:16186124

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