title: Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment.
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.
authors.affiliation: Centro de Diagnóstico de Enfermedades Moleculares (CEDEM), Departamento de Biología Molecular, Centro de Biología Molecular Severo Ochoa (CSIC-UAM), U-746 Centro de Investigación Biomédica en Red de Enfermedades Raras CIBERER-ISCIII, IDIPAZ, Universidad Autónoma de Madrid, Spain.
authors.forename: I
authors.initials: I
authors.lastname: Bravo-Alonso
authors.affiliation: Centro de Diagnóstico de Enfermedades Moleculares (CEDEM), Departamento de Biología Molecular, Centro de Biología Molecular Severo Ochoa (CSIC-UAM), U-746 Centro de Investigación Biomédica en Red de Enfermedades Raras CIBERER-ISCIII, IDIPAZ, Universidad Autónoma de Madrid, Spain.
authors.forename: A
authors.initials: A
authors.lastname: Oyarzabal
authors.affiliation: Departamento de Biología Molecular, Centro de Biología Molecular Severo Ochoa, (CSIC-UAM), U-713 Centro de Investigación Biomédica en Red de Enfermedades Raras CIBERER-ISCIII, Instituto de Investigación Hospital 12 de Octubre, Universidad Autónoma de Madrid, Spain.
authors.forename: M
authors.initials: M
authors.lastname: Sánchez-Aragó
authors.affiliation: Servicio de Microscopía Electrónica, Centro de Biología Molecular Severo Ochoa, Consejo Superior de Investigaciones Científicas, Universidad Autónoma de Madrid, Madrid, Spain.
authors.forename: M T
authors.initials: MT
authors.lastname: Rejas
authors.affiliation: Centro de Diagnóstico de Enfermedades Moleculares (CEDEM), Departamento de Biología Molecular, Centro de Biología Molecular Severo Ochoa (CSIC-UAM), U-746 Centro de Investigación Biomédica en Red de Enfermedades Raras CIBERER-ISCIII, IDIPAZ, Universidad Autónoma de Madrid, Spain.
authors.forename: B
authors.initials: B
authors.lastname: Merinero
authors.affiliation: Department of Neurology, Hospital Sant Joan de Déu (HSJD), U-703 CIBER de Enfermedades Raras (CIBERER), Barcelona, Spain.
authors.forename: A
authors.initials: A
authors.lastname: García-Cazorla
authors.affiliation: Department of Biochemistry, Hospital Sant Joan de Déu (HSJD), U-703 CIBER de Enfermedades Raras (CIBERER), Barcelona, Spain.
authors.forename: R
authors.initials: R
authors.lastname: Artuch
authors.affiliation: Centro de Diagnóstico de Enfermedades Moleculares (CEDEM), Departamento de Biología Molecular, Centro de Biología Molecular Severo Ochoa (CSIC-UAM), U-746 Centro de Investigación Biomédica en Red de Enfermedades Raras CIBERER-ISCIII, IDIPAZ, Universidad Autónoma de Madrid, Spain.
authors.forename: M
authors.initials: M
authors.lastname: Ugarte
authors.affiliation: Centro de Diagnóstico de Enfermedades Moleculares (CEDEM), Departamento de Biología Molecular, Centro de Biología Molecular Severo Ochoa (CSIC-UAM), U-746 Centro de Investigación Biomédica en Red de Enfermedades Raras CIBERER-ISCIII, IDIPAZ, Universidad Autónoma de Madrid, Spain.
authors.forename: P
authors.initials: P
authors.lastname: Rodríguez-Pombo
keywords: BCKDK interference; Bioenergetics profile; Branched-chain α-ketoacid dehydrogenase; Unrestrained branched-chain amino acids catabolism