title: Nitric oxide synthase is up-regulated in muscle fibers in muscular dystrophy.
abstract: Nitric oxide (NO) mediates fundamental physiological actions on skeletal muscle. The neuronal NO synthase isoform (NOS1) was reported to be located exclusively in the sarcolemma. Its loss from the sarcolemma was associated with development of Duchenne muscular dystrophy (DMD). However, new studies evidence that all three NOS isoforms-NOS1, NOS2, and NOS3-are co-expressed in the sarcoplasm both in normal and in DMD skeletal muscles. To address this controversy, we assayed NOS expression in DMD myofibers in situ cytophotometrically and found NOS expression in DMD myofibers up-regulated. These results support the hypothesis that NO deficiency with consequent muscle degeneration in DMD results from NO scavenging by superoxides rather than from reduced NOS expression.
authors.affiliation: Institute of Anatomy, University of Leipzig, Leipzig, Germany.
authors.forename: Karla
authors.initials: K
authors.lastname: Punkt
authors.forename: Stefan
authors.initials: S
authors.lastname: Schering
authors.forename: Sabine
authors.initials: S
authors.lastname: Löffler
authors.forename: Evgeny A
authors.initials: EA
authors.lastname: Minin
authors.forename: Vera E
authors.initials: VE
authors.lastname: Samoilova
authors.forename: Martin
authors.initials: M
authors.lastname: Hasselblatt
authors.forename: Werner
authors.initials: W
authors.lastname: Paulus
authors.forename: Ursula
authors.initials: U
authors.lastname: Müller-Werdan
authors.forename: Uta
authors.initials: U
authors.lastname: Demus
authors.forename: Gabriele
authors.initials: G
authors.lastname: Koehler
authors.forename: Werner
authors.initials: W
authors.lastname: Boecker
authors.forename: Igor B
authors.initials: IB
authors.lastname: Buchwalow
mesh_terms: D001706:Biopsy; D002675:Child, Preschool; D006801:Humans; D007150:Immunohistochemistry; D018485:Muscle Fibers, Skeletal / Q000201:enzymology / Q000473:pathology; D018482:Muscle, Skeletal / Q000201:enzymology* / Q000473:pathology; D009136:Muscular Dystrophies / Q000201:enzymology* / Q000473:pathology; D019001:Nitric Oxide Synthase / Q000096:biosynthesis*; D015854:Up-Regulation
chemical_list: D019001:Nitric Oxide Synthase