1ozu

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[[Image:1ozu.jpg|left|200px]]
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{{STRUCTURE_1ozu| PDB=1ozu | SCENE= }}
{{STRUCTURE_1ozu| PDB=1ozu | SCENE= }}
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'''Crystal Structure of Familial ALS Mutant S134N of human Cu,Zn Superoxide Dismutase (CuZnSOD) to 1.3A resolution'''
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===Crystal Structure of Familial ALS Mutant S134N of human Cu,Zn Superoxide Dismutase (CuZnSOD) to 1.3A resolution===
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==Overview==
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Mutations in the SOD1 gene cause the autosomal dominant, neurodegenerative disorder familial amyotrophic lateral sclerosis (FALS). In spinal cord neurons of human FALS patients and in transgenic mice expressing these mutant proteins, aggregates containing FALS SOD1 are observed. Accumulation of SOD1 aggregates is believed to interfere with axonal transport, protein degradation and anti-apoptotic functions of the neuronal cellular machinery. Here we show that metal-deficient, pathogenic SOD1 mutant proteins crystallize in three different crystal forms, all of which reveal higher-order assemblies of aligned beta-sheets. Amyloid-like filaments and water-filled nanotubes arise through extensive interactions between loop and beta-barrel elements of neighboring mutant SOD1 molecules. In all cases, non-native conformational changes permit a gain of interaction between dimers that leads to higher-order arrays. Normal beta-sheet-containing proteins avoid such self-association by preventing their edge strands from making intermolecular interactions. Loss of this protection through conformational rearrangement in the metal-deficient enzyme could be a toxic property common to mutants of SOD1 linked to FALS.
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The line below this paragraph, {{ABSTRACT_PUBMED_12754496}}, adds the Publication Abstract to the page
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(as it appears on PubMed at http://www.pubmed.gov), where 12754496 is the PubMed ID number.
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{{ABSTRACT_PUBMED_12754496}}
==Disease==
==Disease==
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[[Category: Amyloid-like linear filament]]
[[Category: Amyloid-like linear filament]]
[[Category: Beta barrel]]
[[Category: Beta barrel]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 04:29:47 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 29 11:38:46 2008''

Revision as of 08:38, 29 July 2008

Image:1ozu.png

Template:STRUCTURE 1ozu

Contents

Crystal Structure of Familial ALS Mutant S134N of human Cu,Zn Superoxide Dismutase (CuZnSOD) to 1.3A resolution

Template:ABSTRACT PUBMED 12754496

Disease

Known disease associated with this structure: Amyotrophic lateral sclerosis, due to SOD1 deficiency OMIM:[147450]

About this Structure

1OZU is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Amyloid-like filaments and water-filled nanotubes formed by SOD1 mutant proteins linked to familial ALS., Elam JS, Taylor AB, Strange R, Antonyuk S, Doucette PA, Rodriguez JA, Hasnain SS, Hayward LJ, Valentine JS, Yeates TO, Hart PJ, Nat Struct Biol. 2003 Jun;10(6):461-7. PMID:12754496

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