title: High levels of mitochondrial DNA deletions in substantia nigra neurons in aging and Parkinson disease.
abstract: Here we show that in substantia nigra neurons from both aged controls and individuals with Parkinson disease, there is a high level of deleted mitochondrial DNA (mtDNA) (controls, 43.3% +/- 9.3%; individuals with Parkinson disease, 52.3% +/- 9.3%). These mtDNA mutations are somatic, with different clonally expanded deletions in individual cells, and high levels of these mutations are associated with respiratory chain deficiency. Our studies suggest that somatic mtDNA deletions are important in the selective neuronal loss observed in brain aging and in Parkinson disease.
authors.affiliation: Mitochondrial Research Group, School of Neurology, Neurobiology and Psychiatry, The Medical School, University of Newcastle upon Tyne, Newcastle upon Tyne, NE2 4HH, UK.
authors.forename: Andreas
authors.initials: A
authors.lastname: Bender
authors.forename: Kim J
authors.initials: KJ
authors.lastname: Krishnan
authors.forename: Christopher M
authors.initials: CM
authors.lastname: Morris
authors.forename: Geoffrey A
authors.initials: GA
authors.lastname: Taylor
authors.forename: Amy K
authors.initials: AK
authors.lastname: Reeve
authors.forename: Robert H
authors.initials: RH
authors.lastname: Perry
authors.forename: Evelyn
authors.initials: E
authors.lastname: Jaros
authors.forename: Joshua S
authors.initials: JS
authors.lastname: Hersheson
authors.forename: Joanne
authors.initials: J
authors.lastname: Betts
authors.forename: Thomas
authors.initials: T
authors.lastname: Klopstock
authors.forename: Robert W
authors.initials: RW
authors.lastname: Taylor
authors.forename: Douglass M
authors.initials: DM
authors.lastname: Turnbull
mesh_terms: D000375:Aging / Q000235:genetics*; D001483:Base Sequence; D004272:DNA, Mitochondrial / Q000235:genetics*; D006801:Humans; D010300:Parkinson Disease / Q000235:genetics*; D017384:Sequence Deletion; D013378:Substantia Nigra / Q000473:pathology*
chemical_list: D004272:DNA, Mitochondrial