Article
Aberrant Mitochondrial Fission Is Maladaptive in Desmin Mutation-Induced Cardiac Proteotoxicity.
Journal of the American Heart Association - 9 Jul 2018
Alam Shafiul, Abdullah Chowdhury S, Aishwarya Richa, Miriyala Sumitra, Panchatcharam Manikandan, Peretik Jonette M, Orr A Wayne, James Jeanne, Robbins Jeffrey, Bhuiyan Md Shenuarin
Abstract excerpt
BACKGROUND: Desmin filament proteins interlink the contractile myofibrillar apparatus with mitochondria, nuclei and the sarcolemma. Mutations in the human desmin gene cause cardiac disease, remodeling, and heart failure but the pathophysiological mechanisms remain unknown. METHODS AND RESULTS: Cardiomyocyte-specific overexpression of mutated desmin (a 7 amino acid deletion R172-E178, D7-Des Tg) causes...
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