Literature DB >> 7682901

Hyaline bodies in skeletal muscle of a patient with a mild chronic nonprogressive congenital myopathy.

C Ceuterick1, J J Martin, C Martens.   

Abstract

Subsarcolemmal hyaline bodies were found in a skeletal muscle biopsy of a 10-year-old boy manifesting mild non-evolutive motor disturbances with frequent falls since early childhood. The hyaline bodies occurred in type I fibers and stained intensely with the myosin ATP-ase reaction at pH 4.2. They immunostained intensely with polyclonal anti-skeletal myosin and monoclonal anti-skeletal fast myosin. Additionally, immunoreactive deposits to anti-desmin were observed at the border of some bodies. At the ultrastructural level, these bodies were not surrounded by a limiting membrane and were only localized in subsarcolemmal areas. They contained moderately dense and disorganized filaments which appeared to be in continuity with thick myosin filaments of intact adjacent myofibrils. They stained negatively for polysaccharides according to the periodic acid-thiosemicarbazide silver proteinate method of Thiéry (1967). Analogies and differences with reported cases in the literature with similar subsarcolemmal deposits and other ultrastructural findings in congenital myopathies are discussed.

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Year:  1993        PMID: 7682901

Source DB:  PubMed          Journal:  Clin Neuropathol        ISSN: 0722-5091            Impact factor:   1.368


  3 in total

1.  Myosin storage myopathy mutations yield defective myosin filament assembly in vitro and disrupted myofibrillar structure and function in vivo.

Authors:  Meera C Viswanathan; Rick C Tham; William A Kronert; Floyd Sarsoza; Adriana S Trujillo; Anthony Cammarato; Sanford I Bernstein
Journal:  Hum Mol Genet       Date:  2017-12-15       Impact factor: 6.150

2.  Impaired muscle morphology in a Drosophila model of myosin storage myopathy was supressed by overexpression of an E3 ubiquitin ligase.

Authors:  Martin Dahl-Halvarsson; Montse Olive; Malgorzata Pokrzywa; Michaela Norum; Katarina Ejeskär; Homa Tajsharghi
Journal:  Dis Model Mech       Date:  2020-12-29       Impact factor: 5.758

Review 3.  Myosinopathies: pathology and mechanisms.

Authors:  Homa Tajsharghi; Anders Oldfors
Journal:  Acta Neuropathol       Date:  2012-08-05       Impact factor: 17.088

  3 in total

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