Mitochondrial medicine for neurodegenerative diseases

Int J Biochem Cell Biol. 2010 May;42(5):560-72. doi: 10.1016/j.biocel.2010.01.004. Epub 2010 Jan 11.

Abstract

Mitochondrial dysfunction has been reported in a wide array of neurological disorders ranging from neuromuscular to neurodegenerative diseases. Recent studies on neurodegenerative diseases have revealed that mitochondrial pathology is generally found in inherited or sporadic neurodegenerative diseases and is believed to be involved in the pathophysiological process of these diseases. Commonly seen types of mitochondrial dysfunction in neurodegenerative diseases include excessive free radical generation, lowered ATP production, mitochondrial permeability transition, mitochondrial DNA lesions, perturbed mitochondrial dynamics and apoptosis. Mitochondrial medicine as an emerging therapeutic strategy targeted to mitochondrial dysfunction in neurodegenerative diseases has been proven to be of value, though this area of research is still at in its early stage. In this article, we report on recent progress in the development of several mitochondrial therapies including antioxidants, blockade of mitochondrial permeability transition, and mitochondrial gene therapy as evidence that mitochondrial medicine has promise in the treatment of neurodegenerative diseases.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Antioxidants / metabolism
  • Antioxidants / therapeutic use
  • Apoptosis / drug effects
  • Calcium Signaling / drug effects
  • DNA Damage / drug effects
  • DNA, Mitochondrial / chemistry
  • Drug Delivery Systems
  • Electron Transport Chain Complex Proteins / genetics
  • Electron Transport Chain Complex Proteins / metabolism
  • Genetic Therapy
  • Humans
  • Mitochondria / drug effects
  • Mitochondria / physiology*
  • Mitochondrial Diseases / drug therapy
  • Mitochondrial Diseases / physiopathology*
  • Mitochondrial Membrane Transport Proteins / antagonists & inhibitors
  • Mitochondrial Membrane Transport Proteins / physiology
  • Mitochondrial Permeability Transition Pore
  • Neurodegenerative Diseases / drug therapy
  • Neurodegenerative Diseases / physiopathology*
  • Oxidative Stress / drug effects
  • Oxidative Stress / genetics

Substances

  • Antioxidants
  • DNA, Mitochondrial
  • Electron Transport Chain Complex Proteins
  • Mitochondrial Membrane Transport Proteins
  • Mitochondrial Permeability Transition Pore