Genetic myostatin decrease in the golden retriever muscular dystrophy model does not significantly affect the ubiquitin proteasome system despite enhancing the severity of disease.

作者: Kristine M Wadosky , Joe N Kornegay , Daniel J Bogan , Steven W Cotten , Cam Patterson

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摘要: Recent studies suggest that inhibiting the protein myostatin, a negative regulator of skeletal muscle mass, may improve outcomes in patients with Duchenne muscular dystrophy by enhancing mass. When dystrophin-deficient golden retriever (GRMD) dog was bred whippets having heterozygous mutation for myostatin gene, affected GRMD dogs decreased (GRippets) demonstrated an accelerated physical decline compared to related full myostatin. To examine role ubiquitin proteasome and calpain systems this decline, we determined expression ligases MuRF1, Atrogin-1, RNF25, RNF11, CHIP: subunits PSMA6, PSMB4, PSME1: 1/2 real time PCR cranial sartorius vastus lateralis muscles control, GRMD, GRippet dogs. While individual contributed increased variability seen ligase expression, neither group significantly different from control group. The significant increases caspase-like trypsin-like activity sartorius; however, all three activities did not differ controls. Increased 1 2 groups were identified, but no statistical differences seen. These disease progression which does involve key components involved quality sarcomere other structural proteins.

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