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The activity of the BCKD complex, the rate-limiting enzyme in the BCAA catabolism, has clearly diminished.
The BCKD 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 activity.
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.
Specific BCKD activity was measured upon addition of 0.5 mM [alpha]-ketoisovalerate.
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- Bcl 2
- Bcl 2
- BCL-1 oncogene
- bcl-1, PRAD1
- Bcl-2 Associated Death
- Bcl-2 associating athanogene-1 protein
- Bcl-2 gene