Apr 8, 2016

Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment

Data in Brief
I Bravo-AlonsoP Rodríguez-Pombo

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.

  • References3
  • Citations1

Citations

Mentioned in this Paper

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

Related Feeds

Autism

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.

Related Papers

Clinica Chimica Acta; International Journal of Clinical Chemistry
W Lehnert, E Werle
The Journal of the American Osteopathic Association
J R MarshallM V Kester
Middle East Journal of Anesthesiology
Amir R BaluchJason M Hoover
American Journal of Diseases of Children
R G WESTALL
© 2020 Meta ULC. All rights reserved