PMID- 26365462 OWN - NLM STAT- MEDLINE DCOM- 20160901 LR - 20151027 IS - 0722-5091 (Print) IS - 0722-5091 (Linking) VI - 34 IP - 6 DP - 2015 Nov-Dec TI - Reduced muscle mitochondrial enzyme activity in MuSK-immunized mice. PG - 359-63 LID - 10.5414/NP300875 [doi] AB - AIM: 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. DISCUSSION: Our results suggest that MuSK-immunity might induce muscle atrophy through mitochondrial dysfunction. FAU - Ozkok, Elif AU - Ozkok E FAU - Durmus, Hacer AU - Durmus H FAU - Yetimler, Berrak AU - Yetimler B FAU - Tasli, Hatice AU - Tasli H FAU - Trakas, Nikolaos AU - Trakas N FAU - Ulusoy, Canan AU - Ulusoy C FAU - Lagoumintzis, George AU - Lagoumintzis G FAU - Tzartos, Socrates AU - Tzartos S FAU - Tuzun, Erdem AU - Tuzun E LA - eng PT - Journal Article PL - Germany TA - Clin Neuropathol JT - Clinical neuropathology JID - 8214420 RN - 0 (Autoantibodies) RN - EC 1.3.99.1 (Succinate Dehydrogenase) RN - EC 1.9.3.1 (Electron Transport Complex IV) RN - EC 2.7.- (Phosphotransferases) SB - IM MH - Animals MH - Autoantibodies/immunology MH - Disease Models, Animal MH - Electron Transport Complex IV/*metabolism MH - Mice, Inbred C57BL MH - Mitochondria/*enzymology MH - Muscular Diseases/*enzymology MH - Myasthenia Gravis/*enzymology MH - Phosphotransferases/*immunology MH - Succinate Dehydrogenase/*metabolism EDAT- 2015/09/15 06:00 MHDA- 2016/09/02 06:00 CRDT- 2015/09/15 06:00 PHST- 2015/10/26 00:00 [accepted] PHST- 2015/09/15 06:00 [entrez] PHST- 2015/09/15 06:00 [pubmed] PHST- 2016/09/02 06:00 [medline] AID - 13716 [pii] AID - 10.5414/NP300875 [doi] PST - ppublish SO - Clin Neuropathol. 2015 Nov-Dec;34(6):359-63. doi: 10.5414/NP300875.