Metabolic rerouting of valine and isoleucine oxidation increases survival in zebrafish models of disorders of propionyl-CoA metabolism.
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DOI: 10.1093/hmg/ddaf100
Abstract: Branched-chain amino acid (BCAA) oxidation is a multistep process leading to the formation of acetyl-CoA and propionyl-CoA. The syndromes associated with disturbed BCAA oxidation are clinically and biochemically heterogenous. While the common organic acidemias, propionic… read more here.
Keywords: isoleucine oxidation; mmut; propionyl coa; valine isoleucine ... See more keywords