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