The role of nitric oxide in muscle fibers with oxidative phosphorylation defects
- Department of Neurology, Escola Paulista de Medicina, Universidade Federal de Sao Paulo, Sao Paulo (Brazil)
- Department of Pharmacology, Escola Paulista de Medicina, Universidade Federal de Sao Paulo, Sao Paulo (Brazil)
- Department of Pathology, Escola Paulista de Medicina, Universidade Federal de Sao Paulo, Sao Paulo (Brazil)
NO has been pointed as an important player in the control of mitochondrial respiration, especially because of its inhibitory effect on cytochrome c oxidase (COX). However, all the events involved in this control are still not completely elucidated. We demonstrate compartmentalized abnormalities on nitric oxide synthase (NOS) activity on muscle biopsies of patients with mitochondrial diseases. NOS activity was reduced in the sarcoplasmic compartment in COX deficient fibers, whereas increased activity was found in the sarcolemma of fibers with mitochondrial proliferation. We observed increased expression of neuronal NOS (nNOS) in patients and a correlation between nNOS expression and mitochondrial content. Treatment of skeletal muscle culture with an NO donor induced an increase in mitochondrial content. Our results indicate specific roles of NO in compensatory mechanisms of muscle fibers with mitochondrial deficiency and suggest the participation of nNOS in the signaling process of mitochondrial proliferation in human skeletal muscle.
- OSTI ID:
- 20991476
- Journal Information:
- Biochemical and Biophysical Research Communications, Vol. 359, Issue 3; Other Information: DOI: 10.1016/j.bbrc.2007.05.184; PII: S0006-291X(07)01173-4; Copyright (c) 2007 Elsevier Science B.V., Amsterdam, The Netherlands, All rights reserved; Country of input: International Atomic Energy Agency (IAEA); ISSN 0006-291X
- Country of Publication:
- United States
- Language:
- English
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