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Parkinson's disease-associated pathogenic VPS35 mutation causes complex I deficits.

Leping Zhou1, Wenzhang Wang2, Charles Hoppel3

  • 1Department of Neurology and Institute of Neurology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China; Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA.

Biochimica Et Biophysica Acta. Molecular Basis of Disease
|August 3, 2017
PubMed
Summary

Parkinson's disease (PD) involves mitochondrial complex I defects. Pathogenic VPS35 mutations cause excessive mitochondrial fragmentation, impairing complex I assembly and leading to energy deficits in PD.

Keywords:
Blue native gel electrophoresisComplex I deficitsMitochondrial fissionParkinson's diseaseVPS35

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Area of Science:

  • Biochemistry
  • Cell Biology
  • Neuroscience

Background:

  • Mitochondrial complex I dysfunction is a hallmark of Parkinson's disease (PD) pathogenesis.
  • Mutations in vacuolar protein sorting 35 (VPS35) are linked to autosomal dominant PD.
  • Previous work showed VPS35 mutations induce mitochondrial damage via fragmentation.

Purpose of the Study:

  • To investigate the impact of pathogenic VPS35 mutations on mitochondrial complex I activity.
  • To elucidate the underlying mechanisms connecting VPS35 mutations, mitochondrial dynamics, and complex I function.

Main Methods:

  • Enzymatic activity assays for mitochondrial complex I and II.
  • Analysis of protein expression and assembly of respiratory chain complexes and supercomplexes.
  • Assessment of mitochondrial morphology and the effect of inhibiting mitochondrial fission.

Main Results:

  • VPS35 D620N mutation decreased complex I and II enzymatic activity and respiratory function in patient fibroblasts.
  • Reduced levels of assembled complex I, complex II, and supercomplexes were observed in D620N fibroblasts.
  • Inhibiting mitochondrial fission ameliorated the assembly defects and functional deficits in complex I and II.

Conclusions:

  • Pathogenic VPS35 D620N mutation leads to excessive mitochondrial fission.
  • This fission disrupts the assembly of mitochondrial complex I and supercomplexes.
  • These disruptions result in bioenergetic deficits, contributing to Parkinson's disease pathogenesis.