Contribution of brain intrinsic branched-chain amino acid metabolism in a novel mouse model of maple syrup urine disease
Amanda C. Kuhs,
Laura Ohl,
Tegan Thurston
et al.
Abstract:Maple syrup urine disease (MSUD) results from loss of branched-chain ketoacid dehydrogenase (BCKDH) activity, the committed, rate-limiting step of branched-chain amino acid (BCAA) oxidation. Current treatments, including a low protein diet and liver transplantation, improve peripheral biochemistry and limit episodes of metabolic decompensation but do not fully prevent chronic neuropsychiatric symptoms. The mechanisms underlying chronic neurologic phenotypes remain poorly understood. Currently available MSUD mo… Show more
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