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Signs of early cellular dysfunction in multiple system atrophy.

M Herrera-Vaquero1, A Heras-Garvin1, F Krismer1

  • 1Division of Neurobiology, Department of Neurology, Medizinische Universitat Innsbruck, Innsbruck, Austria.

Neuropathology and Applied Neurobiology
|September 6, 2020
PubMed
Summary
This summary is machine-generated.

Early cellular dysfunction in Multiple System Atrophy (MSA) involves mitochondrial changes and increased susceptibility to oxidative stress. Nuclear translocation of alpha-synuclein suggests an early cellular stress response preceding neurodegeneration in this α-synucleinopathy.

Keywords:
high resolution respirometryinduced pluripotent stem cellsmitochondriamultiple system atrophyoxidative stressα-synuclein

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Area of Science:

  • Neurodegenerative diseases
  • α-synucleinopathies
  • Cellular biology

Background:

  • Multiple system atrophy (MSA) is a fatal neurodegenerative disease linked to α-synuclein.
  • Intracellular α-synuclein aggregates are implicated in pathogenesis, but early disease mechanisms remain unclear.
  • Studying early disease stages is challenging due to reliance on end-stage tissue.

Purpose of the Study:

  • To investigate early cellular dysfunction in Multiple System Atrophy (MSA) using patient-derived cells.
  • To assess mitochondrial function and response to oxidative stress in MSA neural progenitor cells (NPCs).
  • To examine α-synuclein expression and localization in early-stage MSA models.

Main Methods:

  • Generated induced pluripotent stem cells (iPSCs) from MSA patients and controls.
  • Differentiated iPSCs into neural progenitor cells (NPCs).
  • Assessed mitochondrial morphology/function, oxidative stress response, and α-synuclein expression/localization.

Main Results:

  • MSA-derived NPCs showed increased mitochondrial tubulation but preserved respiration.
  • Exposure to oxidative stress induced excessive reactive oxygen species (ROS) and caspase-3 cleavage in MSA NPCs.
  • Nuclear translocation of α-synuclein was observed in MSA NPCs, without altered SNCA gene expression or intracellular aggregates.

Conclusions:

  • MSA-derived NPCs exhibit early cellular dysfunction, including altered redox homeostasis.
  • Cells are compensated at baseline but show increased susceptibility to exogenous oxidative stress.
  • Nuclear α-synuclein translocation indicates an early cellular stress response preceding neurodegeneration in MSA.