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Mitochondria, oxidative stress and neurodegeneration.
J Neurol Sci. 2012 Nov 15; 322(1-2):254-62.JN

Abstract

Mitochondria are involved in ATP supply to cells through oxidative phosphorylation (OXPHOS), synthesis of key molecules and response to oxidative stress, as well as in apoptosis. They contain many redox enzymes and naturally occurring inefficiencies of oxidative phosphorylation generate reactive oxygen species (ROS). CNS functions depend heavily on efficient mitochondrial function, since brain tissue has a high energy demand. Mutations in mitochondrial DNA (mtDNA), generation and presence of ROS and environmental factors may contribute to energy failure and lead to neurodegenerative diseases. Many rare metabolic disorders have been associated with mitochondrial dysfunction. More than 300 pathogenic mtDNA mutations involve proteins that regulate OXPHOS and mitochondrial structural integrity, and have also been described in neurodegenerative diseases with autosomal inheritance. Mitochondria may have an important role in ageing-related neurodegenerative disorders like Parkinson's disease (PD), Alzheimer's disease (AD), Huntington's disease (HD) and amyotrophic lateral sclerosis (ALS). In primary mitochondrial and neurodegenerative disorders, there is strong evidence that mitochondrial dysfunction occurs early and has a primary role in pathogenesis. In the present review, we discuss several mitochondrial diseases as models of neurodegeneration.

Authors+Show Affiliations

Department of Neurological, Neurosurgical and Behavioural Sciences, Medical School, University of Siena, Italy. federico@unisi.itNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

22669122

Citation

Federico, Antonio, et al. "Mitochondria, Oxidative Stress and Neurodegeneration." Journal of the Neurological Sciences, vol. 322, no. 1-2, 2012, pp. 254-62.
Federico A, Cardaioli E, Da Pozzo P, et al. Mitochondria, oxidative stress and neurodegeneration. J Neurol Sci. 2012;322(1-2):254-62.
Federico, A., Cardaioli, E., Da Pozzo, P., Formichi, P., Gallus, G. N., & Radi, E. (2012). Mitochondria, oxidative stress and neurodegeneration. Journal of the Neurological Sciences, 322(1-2), 254-62. https://doi.org/10.1016/j.jns.2012.05.030
Federico A, et al. Mitochondria, Oxidative Stress and Neurodegeneration. J Neurol Sci. 2012 Nov 15;322(1-2):254-62. PubMed PMID: 22669122.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Mitochondria, oxidative stress and neurodegeneration. AU - Federico,Antonio, AU - Cardaioli,Elena, AU - Da Pozzo,Paola, AU - Formichi,Patrizia, AU - Gallus,Gian Nicola, AU - Radi,Elena, Y1 - 2012/06/04/ PY - 2012/03/05/received PY - 2012/05/10/revised PY - 2012/05/12/accepted PY - 2012/6/7/entrez PY - 2012/6/7/pubmed PY - 2013/4/12/medline SP - 254 EP - 62 JF - Journal of the neurological sciences JO - J Neurol Sci VL - 322 IS - 1-2 N2 - Mitochondria are involved in ATP supply to cells through oxidative phosphorylation (OXPHOS), synthesis of key molecules and response to oxidative stress, as well as in apoptosis. They contain many redox enzymes and naturally occurring inefficiencies of oxidative phosphorylation generate reactive oxygen species (ROS). CNS functions depend heavily on efficient mitochondrial function, since brain tissue has a high energy demand. Mutations in mitochondrial DNA (mtDNA), generation and presence of ROS and environmental factors may contribute to energy failure and lead to neurodegenerative diseases. Many rare metabolic disorders have been associated with mitochondrial dysfunction. More than 300 pathogenic mtDNA mutations involve proteins that regulate OXPHOS and mitochondrial structural integrity, and have also been described in neurodegenerative diseases with autosomal inheritance. Mitochondria may have an important role in ageing-related neurodegenerative disorders like Parkinson's disease (PD), Alzheimer's disease (AD), Huntington's disease (HD) and amyotrophic lateral sclerosis (ALS). In primary mitochondrial and neurodegenerative disorders, there is strong evidence that mitochondrial dysfunction occurs early and has a primary role in pathogenesis. In the present review, we discuss several mitochondrial diseases as models of neurodegeneration. SN - 1878-5883 UR - https://www.unboundmedicine.com/medline/citation/22669122/Mitochondria_oxidative_stress_and_neurodegeneration_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0022-510X(12)00258-4 DB - PRIME DP - Unbound Medicine ER -