Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment

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• 作者：I. Bravo-Alonso^a ; A. Oyarzabal^a ; M. Sá ; nchez-Aragó ; ^b ; M.T. Rejas^c ; B. Merinero^a ; A. Garcí ; a-Cazorla^d ; R. Artuch^e ; M. Ugarte^a ; P. Rodrí ; guez-Pombo^a ; ^{mprodriguez@cbm.csic.es" class="auth_mail" title="E-mail the corresponding author}
• 关键词：BCKDK interference ; Branched-chain α-ketoacid dehydrogenase ; Bioenergetics profile ; Unrestrained branched-chain amino acids catabolism
• 刊名：Data in Brief
• 出版年：2016
• 出版时间：June 2016
• 年：2016
• 卷：7
• 期：Complete
• 页码：755-759
• 全文大小：411 K

文摘

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.