title: Reduced muscle mitochondrial enzyme activity in MuSK-immunized mice.
abstract: OBJECTIVE
  Muscle specific kinase (MuSK) antibody-positive myasthenia gravis(MG) patients might present with clinical and electrophysiological signs of muscle atrophy. In this study, we investigated the potential contribution of mitochondrial dysfunction to muscle atrophy induced by MuSK immunity.
  
  
  METHODS
  Mitochondrial enzyme expression was investigated in muscle samples of MuSK-immunized, acetylcholine receptor (AChR)-immunized, and complete Freund's adjuvant (CFA)-immunized C57BL/6 (B6) mice using histochemical methods. Mitochondrial enzyme activity was also investigated in MuSK- and CFA-immunized mice.
  
  
  RESULTS
  Histochemical analysis showed normal muscle fiber activity on succinate dehydrogenase (SDH) and cytochrome oxidase (COX) stains in all immunization groups. However, MuSK-immunized mice had more ragged-red fibers on modified Gomori-trichrome (MGT) stain and more pronounced type 1 muscle fiber atrophy. MuSK-immunized mice also showed reduced citrate synthase, SDH, and NADH-cytochrome c-reductase activity.
  
  
  CONCLUSIONS
  Our results suggest that MuSK-immunity might induce muscle atrophy through mitochondrial dysfunction.
authors.forename: Elif
authors.initials: E
authors.lastname: Özkök
authors.forename: Hacer
authors.initials: H
authors.lastname: Durmuş
authors.forename: Berrak
authors.initials: B
authors.lastname: Yetimler
authors.forename: Hatice
authors.initials: H
authors.lastname: Taşlı
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Trakas
authors.forename: Canan
authors.initials: C
authors.lastname: Ulusoy
authors.forename: George
authors.initials: G
authors.lastname: Lagoumintzis
authors.forename: Socrates
authors.initials: S
authors.lastname: Tzartos
authors.forename: Erdem
authors.initials: E
authors.lastname: Tüzün
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000276:immunology; D004195:Disease Models, Animal; D003576:Electron Transport Complex IV / Q000378:metabolism*; D008810:Mice, Inbred C57BL; D008928:Mitochondria / Q000201:enzymology*; D009135:Muscular Diseases / Q000201:enzymology*; D009157:Myasthenia Gravis / Q000201:enzymology*; D010770:Phosphotransferases / Q000276:immunology*; D013385:Succinate Dehydrogenase / Q000378:metabolism*
chemical_list: D001323:Autoantibodies; D013385:Succinate Dehydrogenase; D003576:Electron Transport Complex IV; D010770:Phosphotransferases