Novel mutations that enhance or repress the aggregation potential of SOD1.

Novel mutations that enhance or repress the aggregation potential of SOD1. Research Abstract Details 

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Novel mutations that enhance or repress the aggregation potential of SOD1. Abstract Text:

Uma Krishnan,Marjatta Son,Bhagya Rajendran,Jeffrey L Elliott,

Mutations in SOD1 cause FALS by a gain of function likely related to protein misfolding and aggregation. SOD1 mutations encompass virtually every domain of the molecule, making it difficult to identify motifs important in SOD1 aggregation. Zinc binding to SOD1 is important for structural integrity, and is hypothesized to play a role in mutant SOD1 aggregation. To address this question, we mutated the unique zinc binding sites of SOD1 and examined whether these changes would influence SOD1 aggregation. We generated single and multiple mutations in SOD1 zinc binding residues (H71, H80 and D83) either alone or in combination with an aggregate forming mutation (A4V) known to cause disease. These SOD1 mutants were assayed for their ability to form aggregates.Using an in vitro cellular SOD1 aggregation assay, we show that combining A4V with mutations in non-zinc binding domains (G37R or G85R) increases SOD1 aggregation potential. Mutations at two zinc binding residues (H71G and D83G) also increase SOD1 aggregation potential. However, an H80G mutation at the third zinc binding residue decreases SOD1 aggregation potential even in the context of other aggregate forming SOD1 mutations. These results demonstrate that various mutations have different effects on SOD1 aggregation potential and that the H80G mutation appears to uniquely act as a dominant inhibitor of SOD1 aggregation.

Novel mutations that enhance or repress the aggregation potential of SOD1. Publishing Authors By Initials

U Krishnan,M Son,B Rajendran,JL Elliott,

For similar inorganic chemicals: elements: metals, heavy: zinc research abstracts see: inorganic chemicals: elements: metals, heavy: zinc research

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MEDLINE DATE:

Novel mutations that enhance or repress the aggregation potential of SOD1. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: Molecular and cellular biochemistry

VOLUME: 287

Page Numbers: 201-11

Journal Abbreviation: Mol. Cell. Biochem.

ISSN: 0300-8177

DAY: 1

MONTH: 04

YEAR: 2006

Novel mutations that enhance or repress the aggregation potential of SOD1. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 364456

Novel mutations that enhance or repress the aggregation potential of SOD1. Keywords Mesh Terms:

KEYWORDS: Zinc

MESH TERMS: physiology

Chemical & Substance for Abstract: Novel mutations that enhance or repress the aggregation potential of SOD1. Information

Substance Name: Superoxide Dismutase

Registry Number: EC 1.15.1.1

Grant and Affiliation Information for Novel mutations that enhance or repress the aggregation potential of SOD1.

AFFILIATION: Department of Neurology, University of Texas, Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, Texas, 75390, USA.

Country: Netherlands

AGENCY: United States NINDS

GRANT: NS40911

ACRONYM: NS

MEDLINETA: Mol Cell Biochem

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