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.
Highlights
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
Bravo-Alonso et al / Data in Brief 7 (2016) 755–759 the knockdown together with the potentiality of the BCKDKknockdown to increase the deficient branched-chain α-ketoacid dehydrogenase activity detected in MSUD patients are shown. & 2016 Elsevier Inc
Graph, figure qRT-PCR (LightCyclers480), XF24 Extracellular Flux analyzer (Seahorse Bioscience, Izasa Scientific) Analyzed MSUD-patient’ fibroblasts lentiviral transduced with shRNAs against BCKDK gene qRT-PCR, oxygen consumption ratio in intact cells, radiometric assay
Summary
Lentiviral particles incorporating shRNAs against BCKDK (NM_00588.1) (Mission shRNA, Sigma Aldrich, St., MO) were generated in HEK293T packaging cells by co-transfection of pLKO. plasmid containing shRNA sequences, packing plasmid pCMV-dR8.74, and envelope plasmid pMD2.G (Addgene, Cambridge, MA) using Lipofectamine and Plus reagent (Life Technologies, Carlsbad, CA). Medium containing viral particles (non-target control shRNA -SHC002- or either of the BCKDK shRNAs targeting different sequences of human BCKDK) were removed 48 h after transfection. Infections of MSUD fibroblasts were performed as described in [1]. ShRNA clone information: clone TRC number: TRCN0000199200, TRCN000010196, and TRCN000010183; clone ID: NM_005881.1828s1c1, NM_005881.x-850s1c1, and NM_005881.x-135s1c1. ShRNA clone information: clone TRC number: TRCN0000199200, TRCN000010196, and TRCN000010183; clone ID: NM_005881.1828s1c1, NM_005881.x-850s1c1, and NM_005881.x-135s1c1. http://www.sigmaaldrich.com/catalog/ genes/BCKDK?lang 1⁄4es®ion1⁄4 ES#shRNA
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