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Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment.

I Bravo-Alonso1, A Oyarzabal1, M Sánchez-Aragó2

  • 1Centro 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.

Data in Brief
|April 8, 2016
PubMed
Summary
This summary is machine-generated.

This study investigates the impact of BCKDK gene knockdown on fibroblast oxygen consumption in Maple Syrup Urine Disease (MSUD) patients. Results show potential for BCKDK knockdown to improve deficient branched-chain alpha-ketoacid dehydrogenase activity.

Keywords:
BCKDK interferenceBioenergetics profileBranched-chain α-ketoacid dehydrogenaseUnrestrained branched-chain amino acids catabolism

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Area of Science:

  • Biochemistry
  • Genetics
  • Metabolic Disorders

Background:

  • Maple Syrup Urine Disease (MSUD) is characterized by reduced branched-chain alpha-ketoacid dehydrogenase complex (BCKDHc) activity.
  • BCKDK deficiency is linked to autism and may influence dietary responses.
  • Understanding BCKDK's role is crucial for metabolic disease research.

Purpose of the Study:

  • To investigate the effect of BCKDK gene knockdown on cellular respiration in MSUD patient fibroblasts.
  • To explore the potential of BCKDK knockdown to compensate for reduced BCKDHc activity in MSUD.
  • To provide data complementing research on mitochondrial response to BCKDK deficiency in autism.

Main Methods:

  • Fibroblasts from an MSUD patient were used.
  • BCKDK gene expression was reduced (knockdown).
  • Real-time oxygen consumption rate was measured to assess mitochondrial function.

Main Results:

  • Data demonstrates the effectiveness of BCKDK gene knockdown in MSUD fibroblasts.
  • The study presents oxygen consumption rate data under conditions of BCKDK absence and high branched-chain amino acid (BCAA) concentrations.
  • Findings suggest BCKDK knockdown may enhance the deficient BCKDHc activity observed in MSUD.

Conclusions:

  • BCKDK knockdown influences cellular respiration in MSUD fibroblasts.
  • This data provides insights into the mitochondrial response to BCKDK deficiency.
  • The findings may contribute to understanding dietary responses in BCKDK-related conditions.