Mitochondrial pathobiology in Parkinson's disease and amyotrophic lateral Sclerosis

Research output: Contribution to journalArticle

Abstract

Parkinson's disease (PD) and amyotrophic lateral sclerosis (ALS) are the second and third most common human adult-onset neurodegenerative diseases, respectively, after Alzheimer's disease. They are characterized by prominent age-related neurodegeneration in selectively vulnerable neural systems. Some forms of PD and ALS are inherited, and genes causing these diseases have been identified. Morphological, biochemical, and genetic, as well as cell and animal model, studies reveal that mitochondria could have a role in this neurodegeneration. The functions and properties of mitochondria might render subsets of selectively vulnerable neurons intrinsically susceptible to cellular aging and stress and overlying genetic variations. In PD, mutations in putative mitochondrial proteins have been identified and mitochondrial DNA mutations have been found in neurons in the substantia nigra. In ALS, changes occur in mitochondrial respiratory chain enzymes and mitochondrial cell death proteins. Transgenic mouse models of human neurodegenerative disease are beginning to reveal possible principles governing the biology of selective neuronal vulnerability that implicate mitochondria and the mitochondrial permeability transition pore. This review will present how mitochondrial pathobiology might contribute to neurodegeneration in PD and ALS and could serve as a target for drug therapy.

Original languageEnglish (US)
JournalJournal of Alzheimer's Disease
Volume20
Issue numberSUPPL.2
DOIs
StatePublished - 2010

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Amyotrophic Lateral Sclerosis
Parkinson Disease
Mitochondria
Neurodegenerative Diseases
Neurons
Mutation
Cell Aging
Mitochondrial Proteins
Substantia Nigra
Electron Transport
Mitochondrial DNA
Transgenic Mice
Molecular Biology
Alzheimer Disease
Cell Death
Animal Models
Drug Therapy
Enzymes
Genes
Proteins

Keywords

  • Adenine nucleotide translocator
  • apoptosis
  • cell death
  • cyclophilin D
  • excitotoxicity
  • mitochondrial permeability transition pore
  • motor neuron
  • ppif voltage-dependent anion channel

ASJC Scopus subject areas

  • Psychiatry and Mental health
  • Geriatrics and Gerontology
  • Clinical Psychology

Cite this

Mitochondrial pathobiology in Parkinson's disease and amyotrophic lateral Sclerosis. / Martin, Lee J.

In: Journal of Alzheimer's Disease, Vol. 20, No. SUPPL.2, 2010.

Research output: Contribution to journalArticle

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