This is a more detailed pathway compared to the previous picture.
For MSUD patients, Leucine,
Isoleucine and Valine will be catabolized by Branched-chain
amino acid transaminases (BCAT) to alpha-ketoisocaproic acid, alpha-keto-beta-methylvaleric
acid and alpha-ketoisovaleric acid respectively. However, for the second step
of this pathway, it requires the Mitochondrial BCKAD complex (Branched Complex
Keto Acid Dehydrogenase) to catabolize the three keto acid to Isovaleryl-CoA,
alpha-methylbutyryl-CoA and Isobutyryl-CoA. These will cause the 2 components
(amino acid and keto acid) to accumulate, causing health problems.
These CoAs are actually supposed to be included in this following pathway, producing important compounds such as Acetyl CoA and Succinyl CoA (intermediates in Krebs Cycle).
References:
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