Article
Inhibition of ubiquitin proteasome system rescues the defective sarco(endo)plasmic reticulum Ca2+-ATPase (SERCA1) protein causing Chianina cattle pseudomyotonia.
The Journal of biological chemistry - 28 Nov 2014
Bianchini Elisa, Testoni Stefania, Gentile Arcangelo, Calì Tito, Ottolini Denis, Villa Antonello, Brini Marisa, Betto Romeo, Mascarello Francesco, Nissen Poul, Sandonà Dorianna, Sacchetto Roberta
Abstract excerpt
A missense mutation in ATP2A1 gene, encoding sarco(endo)plasmic reticulum Ca(2+)-ATPase (SERCA1) protein, causes Chianina cattle congenital pseudomyotonia, an exercise-induced impairment of muscle relaxation. Skeletal muscles of affected cattle are characterized by a selective reduction of SERCA1 in sarcoplasmic reticulum membranes. In this study, we provide evidence that the ubiquitin proteasome system is...
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