In vitro evidence consistent with an interaction between wild‐type and mutant SOD1 protein associated with canine degenerative myelopathy

Abstract

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Intrinsic structural vulnerability in the hydrophobic core induces species-specific aggregation of canine SOD1 with degenerative myelopathy–linked E40K mutation

“…The E40K mutation is the most frequently found mutation in DM-affected dogs ( 14 ). Previous studies have shown that cSOD1 E40K forms an enzymatically active dimer, which is prone to form aggregates in the spinal motor neurons of DM-affected dogs and cultured cells, indicating that the E40K mutation acquires toxicity as in hSOD1-linked ALS ( 15 , 16 , 17 ). In contrast, an experimental mutant, hSOD1 E40K , did not form aggregates in cultured cells, indicating that the E40K mutation induces species-specific aggregation of cSOD1 ( 15 ).…”