caa a22 4500 475745345 CHVBK 20180406123511.0 cr unu---uuuuu 170329e20000701xx s 000 0 eng 10.1023/A:1005688901635 doi (NATIONALLICENCE)springer-10.1023/A:1005688901635 Intracellular localization and isoform expression of the voltage-dependent anion channel (VDAC) in normal and dystrophic skeletal muscle [Elektronische Daten] [Roberto Massa, Lionel Marlier, Alessandro Martorana, Simona Cicconi, Daniela Pierucci, Patrizia Giacomini, Vito de Pinto, Loriana Castellani] Voltage-dependent anion channels (VDACs) are a family of pore-forming proteins encoded by different genes, with at least three protein products expressed in mammalian tissues. The major recognized functional role of VDACs is to permit the almost free permeability of the outer mitochondrial membrane (OMM). Although VDAC1 is the best known among VDAC isoforms, its exclusively mitochondrial location is still debated. Therefore, we have measured its co-localization with markers of cellular organelles or compartments in skeletal muscle fibers by single or double immunofluorescence and traditional as well as confocal microscopy. Our results show that VDAC1 immunoreactivity corresponds to mitochondria and sarcoplasmic reticulum, while sarcolemmal reactivity, previously reported, was not observed. Since VDAC1 has been suggested to be involved in the control of oxidative phosphorylation, we sought for possible gene regulation of VDAC1, VDAC2 and VDAC3 in skeletal muscle of the dystrophin-deficient mdx mouse, which suffers of an impaired control of energy metabolism. Our results show that, while VDAC1 mRNA and protein and VDAC2 mRNA are normally expressed, VDAC3 mRNA is markedly down-regulated in mdx mouse muscle at different ages (before, during and after the outburst of myofiber necrosis). This finding suggests a possible involvement of VDAC3 expression in the early pathogenic events of the mdx muscular dystrophy. Kluwer Academic Publishers, 2000 Massa Roberto Dipartimento di Neuroscienze, Laboratorio di Medicina Molecolare, Università di Roma Tor Vergata, Roma, Italy aut Marlier Lionel Dipartimento di Medicina Interna, Laboratorio di Medicina Molecolare, Università di Roma Tor Vergata, Roma, Italy aut Martorana Alessandro Dipartimento di Neuroscienze, Laboratorio di Medicina Molecolare, Università di Roma Tor Vergata, Roma, Italy aut Cicconi Simona Dipartimento di Medicina Interna, Laboratorio di Medicina Molecolare, Università di Roma Tor Vergata, Roma, Italy aut Pierucci Daniela Dipartimento di Medicina Interna, Laboratorio di Medicina Molecolare, Università di Roma Tor Vergata, Roma, Italy aut Giacomini Patrizia Istituto di Medicina Sperimentale, Consiglio Nazionale delle Ricerche (IMS-CNR), Roma, Italy aut de Pinto Vito Dipartimento di Scienze Chimiche, Laboratorio di Biochimica e Biologia Molecolare, Università di Catania, Catania, Italy aut Castellani Loriana Dipartimento di Neuroscienze, Laboratorio di Medicina Molecolare, Università di Roma Tor Vergata, Roma, Italy aut Journal of Muscle Research & Cell Motility Kluwer Academic Publishers 21/5(2000-07-01), 433-442 0142-4319 21:5<433 2000 21 10974 https://doi.org/10.1023/A:1005688901635 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1023/A:1005688901635 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Massa Roberto Dipartimento di Neuroscienze, Laboratorio di Medicina Molecolare, Università di Roma Tor Vergata, Roma, Italy aut NATIONALLICENCE 700 1- Marlier Lionel Dipartimento di Medicina Interna, Laboratorio di Medicina Molecolare, Università di Roma Tor Vergata, Roma, Italy aut NATIONALLICENCE 700 1- Martorana Alessandro Dipartimento di Neuroscienze, Laboratorio di Medicina Molecolare, Università di Roma Tor Vergata, Roma, Italy aut NATIONALLICENCE 700 1- Cicconi Simona Dipartimento di Medicina Interna, Laboratorio di Medicina Molecolare, Università di Roma Tor Vergata, Roma, Italy aut NATIONALLICENCE 700 1- Pierucci Daniela Dipartimento di Medicina Interna, Laboratorio di Medicina Molecolare, Università di Roma Tor Vergata, Roma, Italy aut NATIONALLICENCE 700 1- Giacomini Patrizia Istituto di Medicina Sperimentale, Consiglio Nazionale delle Ricerche (IMS-CNR), Roma, Italy aut NATIONALLICENCE 700 1- de Pinto Vito Dipartimento di Scienze Chimiche, Laboratorio di Biochimica e Biologia Molecolare, Università di Catania, Catania, Italy aut NATIONALLICENCE 700 1- Castellani Loriana Dipartimento di Neuroscienze, Laboratorio di Medicina Molecolare, Università di Roma Tor Vergata, Roma, Italy aut NATIONALLICENCE 773 0- Journal of Muscle Research & Cell Motility Kluwer Academic Publishers 21/5(2000-07-01), 433-442 0142-4319 21:5<433 2000 21 10974 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer