The DOI matches its Crossref record; 3 of the 3 references it cites were found in a registry or an index.
Nitric oxide synthase complexed with dystrophin and absent from skeletal muscle sarcolemma in Duchenne muscular dystrophy
Open accessAbstract
Nitric oxide (NO) is synthesized in skeletal muscle by neuronal-type NO synthase (nNOS), which is localized to sarcolemma of fast-twitch fibers. Synthesis of NO in active muscle opposes contractile force. We show that nNOS partitions with skeletal muscle membranes owing to association of nNOS with dystrophin, the protein mutated in Duchenne muscular dystrophy (DMD). The dystrophin complex interacts with an N-terminal domain of nNOS that contains a GLGF motif. mdx mice and humans with DMD evince a selective loss of nNOS protein and catalytic activity from muscle membranes, demonstrating a novel role for dystrophin in localizing a signaling enzyme to the myocyte sarcolemma. Aberrant regulation of nNOS may contribute to preferential degeneration of fast-twitch muscle fibers in DMD.
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Cites
The L-Arginine-Nitric Oxide Pathway
Epstein, F. H., Moncada, S., & Higgs, A. (1993) · New England Journal of Medicine · doi:10.1056/nejm199312303292706 · Cited by 6,678
X chromosome-linked muscular dystrophy (mdx) in the mouse
Bulfield, G., Siller, W. G., Wight, P. A., & Moore, K. J. (1984) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.81.4.1189 · Cited by 1,765
Macrophage cytotoxicity: role for L-arginine deiminase and imino nitrogen oxidation to nitrite
Hibbs, J. B., Taintor, R. R., & Vavrin, Z. (1987) · Science · doi:10.1126/science.2432665 · Cited by 1,527
Cited by
Physiology of Nitric Oxide in Skeletal Muscle
Stamler, J. S., & Meissner, G. (2001) · Physiological Reviews · doi:10.1152/physrev.2001.81.1.209 · Cited by 1,080
Dissecting the Interaction between Nitric Oxide Synthase (NOS) and Caveolin
Garcı́a-Cardeña, G., Martasek, P., Masters, B. S. S., Skidd, P. M., Couet, J., Li, S., Lisanti, M. P., & Sessa, W. C. (1997) · Journal of Biological Chemistry · doi:10.1074/jbc.272.41.25437 · Cited by 821
Endogenous nitric oxide synthesis: Biological functions and pathophysiology
Bredt, D. S. (1999) · Free Radical Research · doi:10.1080/10715769900301161 · Cited by 796
Self-citationA nitric oxide synthase transgene ameliorates muscular dystrophy in mdx mice
Wehling, M., Spencer, M. J., & Tidball, J. G. (2001) · The Journal of Cell Biology · doi:10.1083/jcb.200105110 · Cited by 530
Systemic AAV Micro-dystrophin Gene Therapy for Duchenne Muscular Dystrophy
Duan, D. (2018) · Molecular Therapy · doi:10.1016/j.ymthe.2018.07.011 · Cited by 489
Metadata
- Authors
- Brenman, J. E., Chao, D. S., Xia, H., Aldape, K., & Bredt, D. S.
- Venue
- Cell
- Publisher
- Elsevier BV
- Published
- 1995-09
- Volume
- 82
- Issue
- 5
- Pages
- 743-752
- ISSN
- 0092-8674 (ISSN-L)
- OpenAlex
- W2059155059
- Cited by
- 961
- References
- 3
- FWCI
- 16.93
- Type
- journal article
- Language
- en
- Open access
- bronze
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