Myopathy associated with desmin type intermediate filaments. An immunoelectron microscopic study.
The muscle biopsy performed in a 58-year-old woman with a myopathy involving pelvic girdle and lower limbs displayed unusual intermediate filament aggregates by light and electron microscopy. No cardiac involvement was detected. The filamentous aggregates selective for type 1 fibers were found in subsarcolemmal and intermyofibrillar areas closely related to Z bands. Immunohistochemical study by light and electron microscopy using polyclonal and monoclonal antibodies and avidin-biotin peroxidase method revealed that aggregates consisted of desmin filaments. Desmin positive material was unstained with vimentine antibodies.
Pellissier
J F
JF
Laboratoire de Neuropathologie, Faculté de Médecine, CHU Timone, Marseille, France.
Pouget
J
J
Charpin
C
C
Figarella
D
D
D003599:Cytoskeleton / Q000648:ultrastructure*; D003893:Desmin / Q000378:metabolism*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007382:Intermediate Filaments / Q000378:metabolism / Q000648:ultrastructure*; D008854:Microscopy, Electron; D008875:Middle Aged; D009132:Muscles / Q000378:metabolism / Q000648:ultrastructure*; D009135:Muscular Diseases / Q000378:metabolism / Q000473:pathology*
D003893:Desmin