2024-03-28T20:43:38Zhttp://digital.csic.es/dspace-oai/requestoai:digital.csic.es:10261/1512052022-10-06T11:14:44Zcom_10261_79com_10261_1col_10261_332
Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment
Bravo-Alonso, I
Oyarzábal, Alfonso
Sánchez-Aragó, María
Rejas, M. Teresa
Merinero, Begoña
García-Cazorla, Angels
Artuch, Rafael
Ugarte, Magdalena
Rodríguez-Pombo, Pilar
Centro de Diagnóstico de Enfermedades Moleculares (España)
Fundación Ramón Areces
BCKDK interference
Bioenergetics profile Unrestrainedbranched-chainaminoacids catabolism
Branched-chain α-ketoacid dehydrogenase
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.
2017-06-12T08:54:56Z
2017-06-12T08:54:56Z
2016-03-15
2017-06-12T08:54:56Z
artículo
Data in Brief 7: 755- 759 (2016)
http://hdl.handle.net/10261/151205
10.1016/j.dib.2016.03.038
http://dx.doi.org/10.13039/100008054
27054191
eng
Publisher's version
Sí
https://creativecommons.org/licenses/by/4.0/
openAccess