Disulfide‐mediated oligomerization of mutant Cu/Zn‐superoxide dismutase associated with canine degenerative myelopathy
Yuki Shino,
Norifumi Muraki,
Yui Kobatake
et al.
Abstract: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 mutant cSOD1 aggregates remains unclear. Here, we show that mutant cSOD1 losing a copper and zinc io… Show more
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