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Article

Disulfide-mediated oligomerization of mutant Cu/Zn-superoxide dismutase associated with canine degenerative myelopathy.

Protein science : a publication of the Protein Society - 1 Dec 2024

Shino Yuki, Muraki Norifumi, Kobatake Yui, Kamishina Hiroaki, Kato Ryuichi, Furukawa Yoshiaki

Abstract excerpt

A homozygous E40K mutation in the gene coding canine Cu/Zn-superoxide dismutase (cSOD1) causes degenerative myelopathy (DM) in dogs. A pathological hallmark of DM with the cSOD1 mutation is the aggregation of mutant cSOD1 proteins in neurons. The amino acid substitution E40K disrupts a salt bridge between Glu40 and Lys91 and is considered to destabilize the native state of cSOD1; however, the mechanism by which...

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PMID: 39548731
DOI: 10.1002/pro.5210
PMCID: PMC11568243

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