Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment
2016
Bravo-Alonso, I. | Oyarzabal, A. | Sánchez-Aragó, M. | Rejas, M.T. | Merinero, B. | García-Cazorla, A. | Artuch, R. | Ugarte, M. | Rodríguez-Pombo, P.
This data article contains complementary figures to the research article “Mitochondrial response to the BCKDK-deficiency: some clues to understand the positive dietary response in this form of autism” [1]. Herein we present data relative to the effect of knocking down BCKDK gene on the real time oxygen consumption rate of fibroblasts obtained from a Maple Syrup Urine Disease (MSUD) patient. Interference of BCKDK expression on such cells showing a reduced branched-chain α-ketoacid dehydrogenase (BCKDHc) activity; let us generate a scenario to study the direct effect of BCKDK absence in an environment of high branched-chain amino acids (BCAAs) concentrations. Data relative to the effectiveness of the knockdown together with the potentiality of the BCKDK-knockdown to increase the deficient branched-chain α-ketoacid dehydrogenase activity detected in MSUD patients are also shown.
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