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Title

Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment

AuthorsBravo-Alonso, I; Oyarzábal, Alfonso ; Sánchez-Aragó, María ; Rejas, M. Teresa ; Merinero, Begoña ; García-Cazorla, Angels; Artuch, Rafael; Ugarte, Magdalena ; Rodríguez-Pombo, Pilar
KeywordsBCKDK interference
Bioenergetics profile Unrestrainedbranched-chainaminoacids catabolism
Branched-chain α-ketoacid dehydrogenase
Issue Date15-Mar-2016
CitationData in Brief 7: 755- 759 (2016)
AbstractThis 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.
URIhttp://hdl.handle.net/10261/151205
DOI10.1016/j.dib.2016.03.038
Identifiersdoi: 10.1016/j.dib.2016.03.038
issn: 2352-3409
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