Evaluation of the mitochondrial respiratory Chain and oxidative phosphorylation system using blue native gel electrophoresis

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Abstract

The oxidative phosphorylation (OXPHOS) system consists of five multimeric complexes embedded in the mitochondrial inner membrane. They work in concert to drive the aerobic synthesis of ATP. Mitochondrial and nuclear DNA mutations affecting the accumulation and function of these enzymes are the most common cause of mitochondrial diseases and have also been associated with neurodegeneration and aging. For this reason, several approaches for the assessment of the OXPHOS system enzymes have been progressively developed. Based on methods described elsewhere, the use of blue native gel electrophoresis (BNGE) techniques to routinely assess the OXPHOS system and screen for enzymatic defects in homogenates or mitochondrial preparations from tissues or cultured cells is described here. Curr. Protoc. Hum. Genet. 63:19.4.1-19.4.12.

Original languageEnglish
JournalCurrent Protocols in Human Genetics
Issue numberSUPPL.63
DOIs
StatePublished - Dec 1 2009

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Oxidative Phosphorylation
Electron Transport
Electrophoresis
Gels
Viverridae
Mitochondrial Diseases
Mitochondrial Membranes
Enzymes
Mitochondrial DNA
Cultured Cells
Adenosine Triphosphate

Keywords

  • Blue native gel electrophoresis (BNGE)
  • Electron transport chain
  • Mitochondria
  • OXPHOS

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

Cite this

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abstract = "The oxidative phosphorylation (OXPHOS) system consists of five multimeric complexes embedded in the mitochondrial inner membrane. They work in concert to drive the aerobic synthesis of ATP. Mitochondrial and nuclear DNA mutations affecting the accumulation and function of these enzymes are the most common cause of mitochondrial diseases and have also been associated with neurodegeneration and aging. For this reason, several approaches for the assessment of the OXPHOS system enzymes have been progressively developed. Based on methods described elsewhere, the use of blue native gel electrophoresis (BNGE) techniques to routinely assess the OXPHOS system and screen for enzymatic defects in homogenates or mitochondrial preparations from tissues or cultured cells is described here. Curr. Protoc. Hum. Genet. 63:19.4.1-19.4.12.",
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