2vr7

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{{STRUCTURE_2vr7| PDB=2vr7 | SCENE= }}
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'''CRYSTAL STRUCTURE OF G85R ALS MUTANT OF HUMAN CU,ZN SUPEROXIDE DISMUTASE (CUZNSOD) AT 1.58 A RESOLUTION'''
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===CRYSTAL STRUCTURE OF G85R ALS MUTANT OF HUMAN CU,ZN SUPEROXIDE DISMUTASE (CUZNSOD) AT 1.58 A RESOLUTION===
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==Overview==
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Mutations in the gene encoding human copper-zinc superoxide dismutase (SOD1) cause a dominant form of the progressive neurodegenerative disease amyotrophic lateral sclerosis (ALS). Transgenic mice expressing the human Gly85Arg (G85R) SOD1 variant develop paralytic symptoms concomitant with the appearance of SOD1-enriched proteinaceous inclusions in their neural tissues. The process(es) through which misfiolding or aggregation of G85R SOD1 induce motor neuron toxicity are not understood. Here, we present structures of the human G85R SOD1 variant determined by single crystal X-ray diffraction. Alterations in structure of the metal-binding loop elements relative to the wild type enzyme suggest a molecular basis for the metal ion deficiency of the G85R SOD1 protein observed in the CNS of transgenic mice and in purified recombinant G85R SOD1. These findings support the notion that metal-deficient and/or disulfide-reduced mutant SOD1 species contribute to toxicity in SOD1-linked ALS.
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==About this Structure==
==About this Structure==
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[[Category: Zinc]]
[[Category: Zinc]]
[[Category: Zn superoxide dismutase]]
[[Category: Zn superoxide dismutase]]
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Revision as of 12:02, 27 July 2008

Image:2vr7.png

Template:STRUCTURE 2vr7

CRYSTAL STRUCTURE OF G85R ALS MUTANT OF HUMAN CU,ZN SUPEROXIDE DISMUTASE (CUZNSOD) AT 1.58 A RESOLUTION

Template:ABSTRACT PUBMED 18378676

About this Structure

2VR7 is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Structures of the G85R variant of SOD1 in Familial ALS., Cao X, Antonyuk S, Seetharaman SV, Whitson LJ, Taylor AB, Holloway SP, Strange RW, Doucette PA, Valentine JS, Tiwari A, Hayward LJ, Padua S, Cohlberg JA, Hasnain SS, Hart PJ, J Biol Chem. 2008 Mar 31;. PMID:18378676

Page seeded by OCA on Sun Jul 27 15:02:24 2008

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