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Skin fibroblasts were cultured and the activity of propionyl CoA carboxylase was determined in vitro by incubation with propionate, malonate and [14C] sodium bicarbonate, followed by acidification to release carbon dioxide from unmetabolised bicarbonate, and measurement of the radioactivity remaining in the incubation medium.

Moreover, mitochondrial impairment, increased protein turnover, and capillary regression further exacerbate muscle wasting and metabolic inefficiency