Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment. [artículo]
Por: Sánchez Aragó, María [Instituto de Investigación i+12]
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Colaborador(es): Instituto de Investigación imas12.
Tipo de material: 
ArtículoEditor: Data in brief, 2016Descripción: 7:755-9.Recursos en línea: Acceso libre 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.
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Artículo
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Formato Vancouver:
Bravo Alonso I, Oyarzabal A, Sánchez Aragó M, Rejas MT, Merinero B, García Cazorla A et al. Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment. Data Brief. 2016 Mar 15;7:755-9.
PMID: 27054191
PMC4804226
Contiene 6 referencias
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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