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Título: | Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment |
Autor: | Bravo-Alonso, Irene; Oyarzábal, Alfonso CSIC ORCID; Sánchez-Aragó, María CSIC; Rejas, M. Teresa CSIC ORCID ; Merinero, Begoña CSIC; García-Cazorla, Angels; Artuch, Rafael; Ugarte, Magdalena CSIC ; Rodríguez-Pombo, Pilar CSIC ORCID | Palabras clave: | BCKDK interference Bioenergetics profile Unrestrainedbranched-chainaminoacids catabolism Branched-chain α-ketoacid dehydrogenase |
Fecha de publicación: | 15-mar-2016 | Citación: | Data in Brief 7: 755- 759 (2016) | Resumen: | 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. | URI: | http://hdl.handle.net/10261/151205 | DOI: | 10.1016/j.dib.2016.03.038 | Identificadores: | doi: 10.1016/j.dib.2016.03.038 issn: 2352-3409 |
Aparece en las colecciones: | (CBM) Artículos |
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RejasMT_DatasetReportingBCKDKInterferenceInaBCAA.pdf | 386,73 kB | Adobe PDF | Visualizar/Abrir |
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