BCKD - What does BCKD stand for? The Free Dictionary
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The activity of the BCKD
complex, the rate-limiting enzyme in the BCAA catabolism, has clearly diminished.
complex is composed of four subunits named E1a, E1[beta], E2, and E3, around a cubic core of 24 identical dihydrolipoyl transacylase subunits of E2, encoded by the DBT gene (10).
It presents at any age, depending on residual BCKD
MSUD is caused by a deficiency of the BCKD
gene complex, which catalyses the decarboxylation of the alphaketo acids of leucine, isoleucine, and valine to their respective branched-chain acyl-CoAs.
activity was measured upon addition of 0.5 mM [alpha]-ketoisovalerate.