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dc.contributor.authorBravo-Alonso, I
dc.contributor.authorOyarzabal, A
dc.contributor.authorSánchez-Aragó, M
dc.contributor.authorRejas, M T
dc.contributor.authorMerinero, Begoña
dc.contributor.authorGarcía-Cazorla, A
dc.contributor.authorArtuch, R
dc.contributor.authorUgarte, Magdalena
dc.contributor.authorRodríguez-Pombo, P
dc.identifier.citationData Brief.2016 Jun;(7):755-9
dc.description.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.
dc.subjectBCKDK interference
dc.subjectBioenergetics profile
dc.subjectBranched-chain α-ketoacid dehydrogenase
dc.subjectUnrestrained branched-chain amino acids catabolism
dc.titleDataset reporting BCKDK interference in a BCAA-catabolism restricted environment.
dc.identifier.journalData in brief
dc.pubmedtypeJournal Article
Appears in Collections:Fundaciones e Institutos de Investigación > IIS H. U. La Paz > Artículos

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