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Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment

2016; Elsevier BV; Volume: 7; Linguagem: Inglês

10.1016/j.dib.2016.03.038

2352-3409

Irene Bravo‐Alonso, Alfonso Oyarzábal, María Sánchez‐Aragó, María Teresa Rejas, B. Merinero, Àngels García‐Cazorla, Rafael Artuch, Magdalena Ugarte, Pilar Rodríguez‐Pombo,

Metabolism and Genetic Disorders

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.