Animal models for respiratory chain disease

Trends Mol Med. 2001 Dec;7(12):578-81. doi: 10.1016/s1471-4914(01)02167-0.

Abstract

Elucidation of the pathogenesis in respiratory chain diseases is of great importance for developing specific treatments. The limitations inherent to the use of patient material make studies of human tissues often difficult and the mouse has therefore emerged as a suitable model organism for studies of respiratory chain diseases. In this review, we present an overview of the field and discuss in depth a few examples of animal models reproducing pathology of human disease with primary and secondary respiratory chain involvement.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Respiration / genetics
  • DNA-Binding Proteins*
  • Disease Models, Animal*
  • Friedreich Ataxia / genetics
  • Friedreich Ataxia / pathology
  • Friedreich Ataxia / physiopathology
  • High Mobility Group Proteins
  • Humans
  • Mice
  • Mice, Knockout
  • Mice, Transgenic
  • Mitochondria / genetics
  • Mitochondria / metabolism
  • Mitochondria / pathology
  • Mitochondrial Diseases / genetics*
  • Mitochondrial Diseases / pathology
  • Mitochondrial Diseases / physiopathology*
  • Mitochondrial Proteins*
  • Mutation / genetics
  • Nuclear Proteins / genetics
  • Nuclear Proteins / metabolism
  • Transcription Factors / genetics
  • Transcription Factors / metabolism

Substances

  • DNA-Binding Proteins
  • High Mobility Group Proteins
  • Mitochondrial Proteins
  • Nuclear Proteins
  • TFAM protein, human
  • Tfam protein, mouse
  • Transcription Factors
  • mitochondrial transcription factor A