Absence of nitric oxide synthase I despite the presence of the dystrophin complex in human striated muscle |
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Authors: | Zarko Grozdanovic Tatjana Christova Georg Gosztonyi Holger Mellerowicz Dieter Blottner Reinhart Gossrau |
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Institution: | (1) Department of Anatomy, University Clinic Benjamin Franklin, Konigin-Luise-Strasse 15, D-14195 Berlin, Germany;(2) Department of Anatomy and Histology, Medical University, G. Sofiisky Str. 1, BG-1431 Sofia, Bulgaria;(3) Department of Neuropathology, University Clinic Benjamin Franklin, Hindenburgdamm 30, D-12200 Berlin, Germany;(4) Department of Orthopedics, University Clinic Benjamin Franklin, Clayallee 229, D-14195 Berlin, Germany |
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Abstract: | Summary Recently, it has been shown that in human striated muscle the signalling enzyme, brain-type nitric oxide synthase I (NOS I),
is associated with the sarcolemma and complexes with dystrophin and/or members of the dystrophin complex. In order to find
out whether there exists a regular association between NOS I and the complex, muscle biopsies from patients with various muscle
disorders were analysed by enzyme histochemistry and immunohistochemistry. In patients suffering from Duchenne muscular dystrophy,
and to a lesser extent in those with Becker-type dystrophy, NOS I and dystrophin complex components were absent or drastically
reduced in the sarcolemma region. In other dystrophies, as well as in metabolic and inflammatory myopathies, NOS I and dystrophin
complex constituents were expressed normally, while in the case of neurogenic diseases leading to denervation atrophy and
especially congenital idiopathic clubfoot, the immunohistochemical patterns of the distribution of the dystrophin complex
constituents were normal, but NOS I activity and protein were deficient or dramatically diminished. The results can be interpreted
as indicating that, in general, NOS I targeting to the sarcolemma is dependent on particular members of the dystrophin complex,
such as ·-1 syntrophin, yet the expression and/or positioning of NOS I may be under the control of further factors, probably
of neurogenic origin. NOS I-associated diaphorase may thus be a useful complementary tool in the diagnosis of muscle disorders.
This revised version was published online in November 2006 with corrections to the Cover Date. |
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