title: Low stability of Huntington muscle mitochondria against Ca2+ in R6/2 mice.
abstract: OBJECTIVE
  The aim of the present work was the detection of Mitochondrial dysfunction of Huntington's disease (HD).
  
  
  METHODS
  We investigated muscle and muscle mitochondria of 14- to 16-week-old R6/2 mice in comparison with wild-type mice.
  
  
  RESULTS
  Atrophic fibers, increased fuchsinophilic aggregates, and reduced cytochrome c oxidase (15%) were found in HD muscle. With swelling measurements and Ca2+ accumulation experiments, a decreased stability of HD mitochondria against Ca2+-induced permeability transition was detected. Complex I-dependent respiration of HD mitochondria was more sensitive to inhibition by adding 10 microm Ca2+ than wild-type mitochondria.
  
  
  CONCLUSIONS
  Data suggest that the decreased stability of HD mitochondria against Ca2+ contributes to energetic depression and cell atrophy.
authors.affiliation: KeyNeurotek AG, ZENIT Technology Park, Magdeburg, Germany.
authors.forename: Zemfira Z
authors.initials: ZZ
authors.lastname: Gizatullina
authors.forename: Katrin S
authors.initials: KS
authors.lastname: Lindenberg
authors.forename: Phoebe
authors.initials: P
authors.lastname: Harjes
authors.forename: Ying
authors.initials: Y
authors.lastname: Chen
authors.forename: Christoph M
authors.initials: CM
authors.lastname: Kosinski
authors.forename: Bernhard G
authors.initials: BG
authors.lastname: Landwehrmeyer
authors.forename: Albert C
authors.initials: AC
authors.lastname: Ludolph
authors.forename: Frank
authors.initials: F
authors.lastname: Striggow
authors.forename: Stephan
authors.initials: S
authors.lastname: Zierz
authors.forename: Frank N
authors.initials: FN
authors.lastname: Gellerich
mesh_terms: D000818:Animals; D002118:Calcium / Q000494:pharmacology*; D004195:Disease Models, Animal; D004305:Dose-Response Relationship, Drug; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000235:genetics / Q000378:metabolism* / Q000503:physiopathology; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D008931:Mitochondria, Muscle / Q000187:drug effects*; D009419:Nerve Tissue Proteins / Q000235:genetics; D009687:Nuclear Proteins / Q000235:genetics; D010101:Oxygen Consumption / Q000187:drug effects; D012119:Respiration / Q000187:drug effects; D013997:Time Factors; D018911:Trinucleotide Repeats / Q000235:genetics
chemical_list: C506812:Htt protein, mouse; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; D002118:Calcium