Inclusions in familial cytoplasmic body myopathy are stained by anti-dystrophin antibodies.
We report here for the first time positive anti-dystrophin labelling of inclusions in three cases belonging to the same family affected by familial cytoplasmic body myopathy (CBM). Inclusions are also stained, as reported previously, by anti-actin antibodies. The anti-desmin reaction was negative in the centre of cytoplasmic bodies (CB) but showed an enhancement of staining in the peripheral part. Abnormal sarcoplasmic staining of fibres with CB was also observed with that antibody. Anti-vimentin antibody labelling was negative. At present, the significance of this labelling by anti-dystrophin antibodies is unknown, but will open new fields for further investigations in an attempt to understand CB pathogenesis.
Caron
A
A
Laboratory of Neuropathology, CHU Côte de Nâcre, Caen, France.
Chapon
F
F
Berthelin
C
C
Viader
F
F
Lechevalier
B
B
D000199:Actins / Q000032:analysis; D000906:Antibodies; D003893:Desmin / Q000032:analysis; D016189:Dystrophin / Q000032:analysis* / Q000276:immunology; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology* / Q000648:ultrastructure; D008297:Male; D009132:Muscles / Q000473:pathology* / Q000648:ultrastructure; D009468:Neuromuscular Diseases / Q000235:genetics / Q000473:pathology*; D010375:Pedigree; D014746:Vimentin / Q000032:analysis
D000199:Actins; D000906:Antibodies; D003893:Desmin; D016189:Dystrophin; D014746:Vimentin