Sánchez Aragó, María
Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment. [artículo] - Data in brief, 2016 - 7:755-9.
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.
Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment. [artículo] - Data in brief, 2016 - 7:755-9.
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.
