Nitric oxide synthase is up-regulated in muscle fibers in muscular dystrophy.
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.
Punkt
Karla
K
Institute of Anatomy, University of Leipzig, Leipzig, Germany.
Schering
Stefan
S
Löffler
Sabine
S
Minin
Evgeny A
EA
Samoilova
Vera E
VE
Hasselblatt
Martin
M
Paulus
Werner
W
Müller-Werdan
Ursula
U
Demus
Uta
U
Koehler
Gabriele
G
Boecker
Werner
W
Buchwalow
Igor B
IB
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
D019001:Nitric Oxide Synthase