Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment.
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ABSTRACT:
SUBMITTER: Bravo-Alonso I
PROVIDER: S-EPMC4804226 | biostudies-other | 2016 Jun
REPOSITORIES: biostudies-other
Publications
Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment.
Bravo-Alonso I I Oyarzabal A A Sánchez-Aragó M M Rejas M T MT Merinero B B García-Cazorla A A Artuch R R Ugarte M M Rodríguez-Pombo P P
Data in brief 20160315
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 ( ...[more]
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