Abstract:
:Sarcoplasmic masses contain disorganized myofibrillar material and are a striking feature of myotonic dystrophy. However their significance is still unclear. Using immunocytochemistry we studied the expression of cytoskeletal proteins (desmin and vimentin), dystrophin, markers of myogenic differentiation (foetal myosin, neural cell adhesion molecule, bcl-2, insulin-like growth factor-I, fibroblast growth factor, retinoblastoma protein and myoD1), cell cycle regulators (Cdk2, p16, p27 and p57) and muscle proteases (ubiquitin, micro and m calpain and cathepsin D) in muscle biopsies from four patients with myotonic dystrophy. Sarcoplasmic masses were strongly positive for desmin, neural cell adhesion molecule, bcl-2, insulin-like growth factor I, retinoblastoma protein and p57, weakly positive for dystrophin and p16 and negative for vimentin, fibroblast growth factor, myoD1, Cdk2 and p27. Immunoreactivity for foetal myosin was detected only in a few fibres (< 1%). Our data suggest that the late myogenic differentiation programme is activated in sarcoplasmic masses although these areas do not reach complete maturation.
journal_name
Neuropathol Appl Neurobioljournal_title
Neuropathology and applied neurobiologyauthors
Vattemi G,Tomelleri G,Filosto M,Savio C,Rizzuto N,Tonin Pdoi
10.1111/j.1365-2990.2004.00602.xkeywords:
subject
Has Abstractpub_date
2005-02-01 00:00:00pages
45-52issue
1eissn
0305-1846issn
1365-2990pii
NAN602journal_volume
31pub_type
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journal_title:Neuropathology and applied neurobiology
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journal_title:Neuropathology and applied neurobiology
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journal_title:Neuropathology and applied neurobiology
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更新日期:1993-02-01 00:00:00
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更新日期:2020-10-26 00:00:00
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
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journal_title:Neuropathology and applied neurobiology
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更新日期:1992-06-01 00:00:00
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章,评审
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journal_title:Neuropathology and applied neurobiology
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更新日期:2015-06-01 00:00:00