Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment.
Data Brief
; 7: 755-9, 2016 Jun.
Article
en En
| MEDLINE
| ID: mdl-27054191
ABSTRACT
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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1
Colección:
01-internacional
Base de datos:
MEDLINE
Idioma:
En
Revista:
Data Brief
Año:
2016
Tipo del documento:
Article
País de afiliación:
España