Study of some components of the cytoskeleton in muscular disorders with nonspecific cytoplasmic bodies.
To gain a better understanding of the mechanisms involved in the formation of cytoplasmic bodies (CBs), the immunohistochemical and biochemical features of muscle samples with nonspecific CBs were compared to those previously described by our group in cytoplasmic body myopathy (CBM), a congenital disease in which specific CBs are found. Accordingly, we studied nonspecific CBs found in the muscle biopsies of 15 patients with the following diseases: peripheral neuropathy (n = 5), polymyositis (n = 3), myotonic dystrophy (n = 2), sarcoidosis (n = 1), inclusion body myositis (n = 1), hereditary inclusion body myopathy (n = 1), hypothyroidism (n = 1), and muscle atrophy in a patient with multiple brain infarctions (n = 1). Nonspecific inclusions were stained at their periphery by anti-desmin but not by anti-dystrophin antibodies, except in the case of hypothyroidism in which immunostaining was observed with both antibodies. Biochemical studies showed normal amounts of desmin and phosphorylation pattern (the latter data are available for only four patients) as compared to control specimens. Our results differ from those previously reported in CBM, in which CBs were stained by both antidesmin and anti-dystrophin antibodies and in which a hyperphosphorylation of desmin was found. Hypothyroidism is, thus, the only disease in which nonspecific CBs show the same immunostaining pattern as specific CBs from CBM patients. These findings indicate that CBs may result from different mechanisms, and that one of these mechanisms may be shared by CBM and hypothyroidism.
Caron
A
A
Laboratory of Neuropathology, CHU Côte de Nacre, Caen, France.
Gohel
C
C
Mollaret
K
K
Morello
R
R
Chapon
F
F
D000199:Actins / Q000378:metabolism; D000328:Adult; D000368:Aged; D001706:Biopsy; D015153:Blotting, Western; D003598:Cytoskeletal Proteins / Q000378:metabolism*; D003893:Desmin / Q000378:metabolism; D016189:Dystrophin / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000378:metabolism* / Q000473:pathology*; D008875:Middle Aged; D018485:Muscle Fibers, Skeletal / Q000378:metabolism / Q000473:pathology; D018482:Muscle, Skeletal / Q000378:metabolism / Q000473:pathology*; D009135:Muscular Diseases / Q000378:metabolism / Q000473:pathology*; D009218:Myosins / Q000378:metabolism; D010590:Phalloidine / Q000378:metabolism
D000199:Actins; D003598:Cytoskeletal Proteins; D003893:Desmin; D016189:Dystrophin; D010590:Phalloidine; D009218:Myosins