报告在支链氨基酸分解代谢受限环境中BCKDK干扰的数据集。
Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment.
作者信息
Bravo-Alonso I, Oyarzabal A, Sánchez-Aragó M, Rejas M T, Merinero B, García-Cazorla A, Artuch R, Ugarte M, Rodríguez-Pombo P
机构信息
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.
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.
出版信息
Data Brief. 2016 Mar 15;7:755-9. doi: 10.1016/j.dib.2016.03.038. eCollection 2016 Jun.
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.
本数据文章包含与研究文章《线粒体对BCKDK缺陷的反应：理解这种自闭症形式中积极饮食反应的一些线索》[1]互补的图表。在此，我们展示了敲低BCKDK基因对从枫糖尿症（MSUD）患者获得的成纤维细胞实时耗氧率影响的数据。BCKDK表达对这类显示支链α-酮酸脱氢酶（BCKDHc）活性降低的细胞的干扰；使我们能够构建一个场景，以研究在高支链氨基酸（BCAAs）浓度环境中BCKDK缺失的直接影响。还展示了与敲低有效性相关的数据，以及BCKDK敲低增加MSUD患者中检测到的缺陷支链α-酮酸脱氢酶活性的潜力。
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