Abstract
Myostatin is a negative regulator of muscle mass and strength. Sporadic inclusion-body myositis (s-IBM) is the most common degenerative muscle disease of older persons and is characterized by pronounced muscle wasting. s-IBM is of unknown etiology and pathogenesis, and it lacks definitive treatment. We have now demonstrated in samples from 12 s-IBM biopsies that: (1) by light and electron microscopic immunocytochemistry, myostatin/myostatin precursor is accumulated within muscle fibers and co-localized with amyloid-β (Aβ); (2) by immunoblots, both myostatin and myostatin precursor are increased; and (3) by immunoprecipitation, myostatin precursor complexes with Aβ. Our study suggests that myostatin/myostatin precursor, either alone, or bound to Aβ, may play a novel role in the pathogenesis of s-IBM.
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Acknowledgments
This study was supported by grants (to V.A.) from the National Institutes of Health (AG16768 Merit Award), the Myositis Association, the Muscular Dystrophy Association, and the Helen Lewis Research Fund. S.W. is on leave from the Department of Anatomy and Neurobiology, Medical University of Gdansk, Gdansk, Poland. Maggie Baburyan provided excellent technical assistance in electron microscopy.
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Wójcik, S., Engel, W.K., McFerrin, J. et al. Myostatin is increased and complexes with amyloid-β within sporadic inclusion-body myositis muscle fibers. Acta Neuropathol 110, 173–177 (2005). https://doi.org/10.1007/s00401-005-1035-3
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DOI: https://doi.org/10.1007/s00401-005-1035-3