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  • Title: Histological determination of nitric oxide synthase (NOS) and NADPH-diaphorase in ragged-red fibers from patients with mitochondrial encephalomyopathies.
    Author: Ohkoshi N, Mizusawa H, Fujita T, Shoji S.
    Journal: J Neurol Sci; 1997 Aug; 149(2):151-6. PubMed ID: 9171323.
    Abstract:
    To determine localization of nitric oxide synthase in diseased muscle, we performed immunohistochemical analyses of neuronal-type nitric oxide synthase (nNOS) and endothelial-type nitric oxide synthase (ec-NOS) in biopsied muscles from five patients with mitochondrial encephalomyopathies. Immunostaining of nNOS was prominent in the sarcolemmal region of the ragged-red fibers, and weak in the sarcolemmal region of normal fibers. Immunostaining of ec-NOS was strongly positive in the myofibrils of ragged-red fibers. Ec-NOS immunoreactivity corresponded to fibers positive for SDH by histochemistry. Histochemical methods revealed prominent staining for NADPH-diaphorase on surface membranes of the ragged-red fibers. Findings suggest that nitric oxide is important in the muscles of patients with mitochondrial encephalomyopathies.
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