Apr 8, 2016

Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment

Data in Brief
I Bravo-AlonsoP Rodríguez-Pombo


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.

  • References3
  • Citations1


Mentioned in this Paper

ARID4B wt Allele
Specimen Type - Fibroblasts
Gene Knockdown Techniques
Keto Acids
Maple Syrup Urine Disease
Catabolic Process
2-Methylbutyryl-CoA Dehydrogenase Deficiency

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Autism spectrum disorder is associated with challenges with social skills, repetitive behaviors, and often accompanied by sensory sensitivities and medical issues. Here is the latest research.

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