Skip to main content
SpringerLink
Log in

Decreased total nitric oxide production in patients with duchenne muscular dystrophy

  • Short Communication
  • Published:
Journal of Biomedical Science

Abstract

Plasma nitric oxide (NO) levels in Duchenne muscular dystrophy (DMD) patients were significantly lower than those observed in both healthy controls and in patients with other neuromuscular disorders. The correlation between NO level and ejection fraction was significant (r=−0.384, p=0.0391) in the DMD group. Disruption of NO systems may contribute to the development of muscular dystrophy and have implications for therapeutic strategies.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

References

  1. Azzena GB, Mancinelli R. Nitric oxide regenerates the normal colonic peristaltic activity in mdx dystrophic mouse. Neurosci Lett 261:9–12;1999.

    Article  PubMed  Google Scholar 

  2. Baccari MC, Romagnani P, Calamai F. Impaired nitrergic relaxations in the gastric fundus of dystrophic (mdx) mice. Neurosci Lett 282:105–108;2000.

    Article  PubMed  Google Scholar 

  3. Barohn RJ, Levine EJ, Olson JO, Mendell JR. Gastric hypomotility in Duchenne's muscular dystrophy. N Engl J Med 319:15–18;1988.

    PubMed  Google Scholar 

  4. Bia BL, Cassidy PJ, Young ME, Rafael JA, Leighton B, Davies KE, Radda GK, Clarke K. Decreased myocardial nNOS, increased iNOS and abnormal ECGs in mouse models of Duchenne muscular dystrophy. J Mol Cell Cardiol 31:1857–1862;1999.

    Article  PubMed  Google Scholar 

  5. Blake DJ, Kroger S. The neurobiology of Duchenne muscular dystrophy: Learning lessons from muscle? Trends Neurosci 23:92–99;2000.

    Article  PubMed  Google Scholar 

  6. Brenman JE, Chao DS, Xia H, Aldape K, Bredt DS. Nitric oxide synthase complexed with dystrophin and absent from skeletal muscle sarcolemma in Duchenne muscular dystrophy. Cell 82:743–752;1995.

    Article  PubMed  Google Scholar 

  7. Brenman JE, Chao DS, Gee SH, McGee AW, Craven SE, Santillano DR, Wu Z, Huang F, Xia H, Peters MF, Froehner SC, Bredt DS. Interaction of nitric oxide synthase with the postsynaptic density protein PSD-95 and alphal-syntrophin mediated by PDZ domains. Cell 84:757–767;1996.

    Article  PubMed  Google Scholar 

  8. Chang WJ, Iannaccone ST, Lau KS, Masters BSS, McCabe TJ, McMillan K, Padre RC, Spencer MJ, Tidball JG, Stull JT. Neuronal nitric oxide synthase and dystrophin-deficient muscular dystrophy. Proc Natl Acad Sci USA 93:9142–9147;1996.

    Article  PubMed  Google Scholar 

  9. Chao DS, Gorospe JR, Brenman JE, Rafael JA, Peters MF, Froehner SC, Hoffman EP, Chamberlain JS, Bredt DS. Selective loss of sarcolemmal nitric oxide synthase in Becker muscular dystrophy. J Exp Med 184:609–618;1996.

    Article  PubMed  Google Scholar 

  10. Crosbie RH. NO vascular control in Duchenne muscular dystrophy. Nat Med 7:27–29;2001.

    Article  PubMed  Google Scholar 

  11. Engel AG, Banker BQ. Myology: Basic and Clinical. New York, McGraw-Hill, 1994.

    Google Scholar 

  12. Evans HG, Lewis MJ, Shah AM. Interleukin-1-beta modulates myocardial contraction via dexamethasone sensitive production of nitric oxide. Cardiovasc Res 27:1486–1490;1993.

    PubMed  Google Scholar 

  13. Finkel MS, Oddis CV, Jacob TD, Watkins SC, Hattler BG, Simmons RL. Negative inotropic effects of cytokines on the heart mediated by nitric oxide. Science 257:387–389;1992.

    PubMed  Google Scholar 

  14. Gaffney JF, Kingston WJ, Metlay LA, Gramiak R. Left ventricular thrombus and systemic emboli complicating the cardiomyopathy of Duchenne's muscular dystrophy. Arch Neurol 46:1249–1252;1989.

    PubMed  Google Scholar 

  15. Gardiner SM, Compton AM, Bennett T, Palmer RM, Moncada S. Control of regional blood flow by endothelium-derived nitric oxide. Hypertension 15:486–492;1990.

    PubMed  Google Scholar 

  16. Gucuyener K, Ergenekon E, Erbas D, Pinarli G, Serdaroglu A. The serum nitric oxide levels in patients with Duchenne muscular dystrophy. Brain Dev 22:181–183;2000

    Article  PubMed  Google Scholar 

  17. Guslandi M. Nitric oxide: An ubiquitous actor in the gastrointestinal tract. Dig Dis 12:28–36;1994.

    PubMed  Google Scholar 

  18. Hashida-Okumura A, Okumura N, Iwamatsu A, Buijs RM, Romijn HJ, Nagai K. Interaction of neuronal nitric-oxide synthase with alphalsyntrophin in rat brain. J Biol Chem 274:11736–11741;1999.

    Article  PubMed  Google Scholar 

  19. Huang KL, Wu CP, Kang BH, Lin YC. Chronic hypoxia attenuates nitric oxide-dependent hemodynamic responses to acute hypoxia. J Biomed Sci 9:206–212;2002.

    PubMed  Google Scholar 

  20. Kobzik L, Reid MB, Bredt DS, Stamler JS, Nitric oxide in skeletal muscle. Nature 372:546–548;1994.

    Article  PubMed  Google Scholar 

  21. Lau KS, Grange RW, Chang WJ, Kamm KE, Sarelius I, Stull JT. Skeletal muscle contractions stimulate cGMP formation and attenuate vascular smooth muscle myosin phosphorylation via nitric oxide. FEBS Lett 431:71–74;1998.

    Article  PubMed  Google Scholar 

  22. Lee KH, Baek MY, Moon KY, Song WK, Chung CH, Ha DB, Kang MS. Nitric oxide as a messenger molecule for myoblast fusion. J Biol Chem 269:14371–14374;1994.

    PubMed  Google Scholar 

  23. Leon SH, Schuffler MD, Kettler M, Rohrmann CA. Chronic intestinal pseudoobstruction as a complication of Duchenne's muscular dystrophy. Gastroenterology 90:455–459;1986.

    PubMed  Google Scholar 

  24. Levine B, Kalman J, Mayer L, Fillit HM, Packer M. Elevated circulating levels of tumour necrosis factor in severe chronic heart failure. N Eng J Med 323:236–241;1990.

    Google Scholar 

  25. Riggs T. Cardiomyopathy and pulmonary emboli in terminal Duchenne's muscular dystrophy. Am Heart J 119:690–693;1990.

    PubMed  Google Scholar 

  26. Sander M, Chavoshan B, Harris SA, Iannaccone ST, Stull JT, Thomas GD, Victor RG. Functional muscle ischemia in neuronal nitric oxide synthase-deficient skeletal muscle of children with Duchenne muscular dystrophy. Proc Natl Acad Sci USA 97:13818–13823;2000.

    Article  PubMed  Google Scholar 

  27. Schoser BG, Lück G, Blottner D. Partial loss of NADPH-diaphorase/nitric oxide synthase-complex in amyotrophic lateral sclerosis and human type-II myofiber atrophy. Neurosci Lett 231:163–166;1997.

    Article  PubMed  Google Scholar 

  28. Straub V, Ratjen F, Amthor H, Voit T, Grasemann H. Airway nitric oxide in Duchenne muscular dystrophy. J Pediatr 141:132–134;2002.

    Article  PubMed  Google Scholar 

  29. Thomas GD, Sander M, Lau KS, Huang PL, Stull JT, Victor RG. Impaired metabolic modulation of alpha-adrenergic vasoconstriction in dystrophin-deficient skeletal muscle. Proc Natl Acad Sci USA 95:15090–15095;1998.

    Article  PubMed  Google Scholar 

  30. Winlaw DS, Smythe GA, Keogh AM, Schyvens CG, Spratt PM, Macdonald PS. Increased nitric oxide production in heart failure. Lancet 344:373–374;1994.

    Article  PubMed  Google Scholar 

  31. Yotsukura M, Miyagawa M, Tsuya T, Ishihawa T, Ishikawa K. Pulmonary hypertension in progressive muscular dystrophy of the Duchenne type. Jpn Circ J 52:321–326;1988.

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology and Geriatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Sakuragaoka 8-35-1, 890-8520, Kagoshima, Japan

    Takefumi Kasai & Mitsuhiro Osame

  2. Department of Laboratory and Vascular Medicine, Kagoshima University Graduate School of Medical and Dental Sciences, Japan

    Kazuhiro Abeyama, Teruto Hashiguchi & Kuro Maruyama

  3. Department of Neurology, National Minamikyusyu Hospital, Kagoshima, Japan

    Hidetoshi Fukunaga

Authors
  1. Takefumi Kasai
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kazuhiro Abeyama
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Teruto Hashiguchi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Hidetoshi Fukunaga
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Mitsuhiro Osame
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Kuro Maruyama
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kasai, T., Abeyama, K., Hashiguchi, T. et al. Decreased total nitric oxide production in patients with duchenne muscular dystrophy. J Biomed Sci 11, 534–537 (2004). https://doi.org/10.1007/BF02256104

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02256104

Key Words

Search

Navigation