Oxidative stress in inherited mitochondrial diseases

G Hayashi, G Cortopassi - Free Radical Biology and Medicine, 2015 - Elsevier
Mitochondria are a source of reactive oxygen species (ROS). Mitochondrial diseases are the
result of inherited defects in mitochondrially expressed genes. One potential …

Oxidative stress, mitochondrial dysfunction and cellular stress response in Friedreich's ataxia

V Calabrese, R Lodi, C Tonon, V D'Agata… - Journal of the …, 2005 - Elsevier
There is significant evidence that the pathogenesis of several neurodegenerative diseases,
including Parkinson's disease, Alzheimer's disease, Friedreich's ataxia (FRDA), multiple …

Oxidative stress: mechanistic insights into inherited mitochondrial disorders and Parkinson's disease

M Al Shahrani, S Heales, I Hargreaves… - Journal of Clinical …, 2017 - mdpi.com
Oxidative stress arises when cellular antioxidant defences become overwhelmed by a
surplus generation of reactive oxygen species (ROS). Once this occurs, many cellular …

Mitochondrial dysfunction in some oxidative stress-related genetic diseases: Ataxia-Telangiectasia, Down Syndrome, Fanconi Anaemia and Werner Syndrome

FV Pallardó, A Lloret, M Lebel, M d'Ischia, VC Cogger… - Biogerontology, 2010 - Springer
Oxidative stress is a phenotypic hallmark in several genetic disorders characterized by
cancer predisposition and/or propensity to premature ageing. Here we review the published …

[HTML][HTML] Mitochondrial dysfunction in neurodegenerative diseases

MF Beal - Biochimica et Biophysica Acta (BBA)-Bioenergetics, 1998 - Elsevier
A potential pivotal role for mitochondrial dysfunction in neurodegenerative diseases is
gaining increasing acceptance. Mitochondrial dysfunction leads to a number of deleterious …

Oxidative stress and mitochondrial dysfunction in neurodegeneration.

HJ Tritschler, L Packer, R Medori - Biochemistry and molecular …, 1994 - europepmc.org
In the last 4 years much progress has been made in the understanding of mitochondrial
disorders. Point-mutations, deletions and depletion of the mitochondrial genome are …

Mitochondrial DNA mutations and oxidative stress in mitochondrial diseases

YH Wei, HC Lee - Advances in Clinical Chemistry, 2003 - Elsevier
6.2. Point Mutations in tRNA Genes of mtDNA............................. 6.3. Point Mutations in rRNA
Genes of mtDNA............................. 6, 4. Point Mutations in Protein-Coding Genes of …

[HTML][HTML] Mitochondrial involvement in Parkinson's disease, Huntington's disease, hereditary spastic paraplegia and Friedreich's ataxia

AHV Schapira - Biochimica et Biophysica Acta (BBA)-Bioenergetics, 1999 - Elsevier
Respiratory chain dysfunction has been identified in several neurodegenerative disorders.
In Friedreich's ataxia (FA) and Huntington's disease (HD), where the respective mutations …

[HTML][HTML] The epidemiology of mitochondrial disorders—past, present and future

AM Schaefer, RW Taylor, DM Turnbull… - Biochimica et Biophysica …, 2004 - Elsevier
A number of epidemiological studies of mitochondrial disease have been carried out over
the last decade, clearly demonstrating that mitochondrial disorders are far more common …

The impact of reactive oxygen species and genetic mitochondrial mutations in Parkinson's disease

L Zuo, MS Motherwell - Gene, 2013 - Elsevier
The exact pathogenesis of Parkinson's disease (PD) is still unknown and proper
mechanisms that correspond to the disease remain unidentified. It is understood that PD is …