Mitochondrial disease

@article{Schapira2006MitochondrialD,
  title={Mitochondrial disease},
  author={Anthony H V Schapira},
  journal={The Lancet},
  year={2006},
  volume={368},
  pages={70-82}
}
Underlying role of mitochondrial mutagenesis in the pathogenesis of a disease and current approaches for translational research
TLDR
It is suggested that environmental agents can cause damage in mitochondrial DNA and consequently lead to mutagenesis and current approaches for handling mitochondrial diseases, as well as available prenatal diagnostic tests, towards eliminating these maternally inherited diseases.
Mitochondrial Medicine for Aging and Neurodegenerative Diseases
  • P. Reddy
  • Biology
    NeuroMolecular Medicine
  • 2008
TLDR
Critical issues of mitochondria causing dysfunction in aging and neurodegenerative diseases are discussed, and the potential of developing mitochondrial medicine, particularly mitochondrially targeted antioxidants, to treat aging and neurologic diseases is discussed.
Mitochondrial Dysfunction and Oxidative Stress in Parkinson’s Disease
TLDR
Molecular studies in both toxin based models and genetic based models of Parkinson’s disease suggest a major etiologic role for mitochondrial dysfunction in the pathogenesis of PD.
Mitochondrial matters of the brain: the role in Huntington’s disease
TLDR
The localization of mutant htt within mitochondria and the association between transcriptional dysregulation caused by impaired PGC-1α activity with abnormal mitochondrial biogenesis and function has provided further links with additional potential pathogenic mechanisms.
An overview of neurological and neuromuscular signs in mitochondrial diseases.
Genetic variation in PARL influences mitochondrial content
TLDR
Assessment of the influence of variation in the PARL gene on mitochondrial content suggests that genetic variation within PARL influences mitochondrial abundance and integrity.
A genetic view of the mitochondrial role in ageing: killing us softly.
TLDR
Accumulation of mitochondrial mutations with subtle functionality, which was overlooked by the mechanisms of selection, supplemented by slightly affected fusion-fission cycles, will hamper mitochondrial functional complementation within cells, disrupt mito-nuclear interactions and lead to ageing.
...
...