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Mitochondrial quality control: a matter of life and death for neurons

EMBO J. 2012 Mar 21;31(6):1336-49. doi: 10.1038/emboj.2012.38. Epub 2012 Feb 21.

Abstract

Neuronal survival critically depends on the integrity and functionality of mitochondria. A hierarchical system of cellular surveillance mechanisms protects mitochondria against stress, monitors mitochondrial damage and ensures the selective removal of dysfunctional mitochondrial proteins or organelles. Mitochondrial proteases emerge as central regulators that coordinate different quality control (QC) pathways within an interconnected network of mechanisms. A failure of this system causes neuronal loss in a steadily increasing number of neurodegenerative disorders, which include Parkinson's disease, spinocerebellar ataxia, spastic paraplegia and peripheral neuropathies. Here, we will discuss the role of the mitochondrial QC network for neuronal survival and neurodegeneration.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Apoptosis / physiology*
  • Cell Survival / physiology*
  • Humans
  • Mitochondria / metabolism*
  • Mitochondrial Proteins / metabolism
  • Neurodegenerative Diseases / metabolism
  • Neurodegenerative Diseases / pathology
  • Neurons / cytology*
  • Neurons / metabolism
  • Neurons / pathology
  • Peptide Hydrolases / metabolism

Substances

  • Mitochondrial Proteins
  • Peptide Hydrolases