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- W2755914902 abstract "GNE myopathy is a debilitating autosomal recessive myopathy due to mutations in the GNE gene, UDP-N-acetylglucosamine-2-epimerase/ N-Acetylmannosamine kinase, which encodes critical enzymes for sialic acid biosynthesis. Clinically, it is characterized by atrophy and weakness in distal skeletal muscle. We have established that a key factor in the mechanism of disease is sialic acid deficiency, a target for recent and ongoing clinical trials. In GNE myopathy patients with existing severe muscle atrophy and degeneration, however, exploring additional therapeutic options may prove beneficial. In this study, we demonstrated the efficacy of myostatin pathway blockade, which promotes muscle growth, on GNE myopathy model mice. CDD866, a murinized anti-activin type II receptor antibody, not only promoted body weight gain and increase in muscle size, but also led to the recovery of specific tetanic force, which is a functional indicator of muscle contractile devices. In addition, we demonstrated that increased S-nitrosylation of contractile and metabolic proteins leads to muscle weakness in GNE myopathy mice. More importantly, CDD866 treatment in mice reversed S-nitrosylation of the muscle proteins along with the recovery of muscle function. Our results suggest that protein synthesis via myostatin pathway blockade has a beneficial effect by providing newly-synthesized fresh muscle proteins to chronic myopathic muscles in addition to muscle size increment. Myostatin pathway blockade may be a promising therapy on GNE myopathy in addition to sialic acid supplementation." @default.
- W2755914902 created "2017-09-25" @default.
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- W2755914902 date "2017-10-01" @default.
- W2755914902 modified "2023-09-25" @default.
- W2755914902 title "Muscle growth by activin type II receptor blocking ameliorates weakness in GNE myopathy mice" @default.
- W2755914902 doi "https://doi.org/10.1016/j.nmd.2017.06.489" @default.
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