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Different Metabolic Properties of Mitochondrial Oxidative Phosphorylation in Different Cell Types ‐ Important Implications for Mitochondrial Cytopathies
Author(s) -
Kunz Wolfram S.
Publication year - 2003
Publication title -
experimental physiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.925
H-Index - 101
eISSN - 1469-445X
pISSN - 0958-0670
DOI - 10.1113/eph8802512
Subject(s) - oxidative phosphorylation , mitochondrial dna , mitochondrion , biology , gene isoform , microbiology and biotechnology , respiratory chain , phosphorylation , mitochondrial respiratory chain , biochemistry , gene
The metabolic and functional diversity of animal mitochondria caused by different mitochondrial compositions due to tissue‐specific mitochondrial pathways and tissue‐specific differences in expression of isoforms of subunits of enzymes participating in oxidative phosphorylation will be reviewed here. Applying the concept of metabolic control analysis, the relevance of this diversity for the explanation of tissue‐specific effects observed in mitochondrial diseases with homoplasmic mitochondrial DNA mutations and nuclear‐encoded respiratory chain defects is discussed.