Expression of late myogenic differentiation markers in sarcoplasmic masses of patients with myotonic dystrophy.
Neuropathol Appl Neurobiol
; 31(1): 45-52, 2005 Feb.
Article
in En
| MEDLINE
| ID: mdl-15634230
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.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Cell Differentiation
/
Muscle, Skeletal
/
Muscle Cells
/
Myotonic Dystrophy
Limits:
Adult
/
Humans
/
Middle aged
Language:
En
Journal:
Neuropathol Appl Neurobiol
Year:
2005
Document type:
Article
Affiliation country:
Italy