1oez
From Proteopedia
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ZN HIS46ARG MUTANT OF HUMAN CU, ZN SUPEROXIDE DISMUTASE
Overview
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. ... [(full description)]
About this Structure
1OEZ is a [Single protein] structure of sequence from [Homo sapiens] with ZN and SO4 as [ligands]. Active as [[1]], with EC number [1.15.1.1]. 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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