Altered distribution of lamin and emerin in muscle nuclei of sIBM patients.
OBJECTIVE
Sporadic inclusion body myositis (sIBM) is a chronic acquired inflammatory myopathy. The cause of sIBM remains unknown and its pathogenesis is controversial. There is a hypothesis [Karpati and Carpenter 1993] that the rimmed vacuoles result from nuclear breakdown, and IBM filaments are formed from components of the nuclear matrix.


METHODS
For nuclear membrane protein detection, six IBM patients were studied using immunohistochemical and immunochemical techniques.


RESULTS
It was demonstrated that in the interior of 10-15% myonuclei emerin and lamin A/C inclusions appeared constantly. This finding indicated an abberant localization of lamin A/C epitopes, the presence of presumptive lamin A (67 KDu) and emerin as in the affected nuclei.


CONCLUSIONS
We support the suggestion that truncated, changed lamin A protein takes part in nuclear disintegration and rimmed vacuole formation.
Fidzianska
A
A
Neuromuscular Unit, Medical Research Center, Polish Academy of Sciences, Pawinskiego 5 str., 02-106 Warsaw, Poland. neurmyol@cmdik.pan.pl
Glinka
Z
Z
Kaminska
A
A
Niebroj-Dobosz
I
I
D000368:Aged; D016022:Case-Control Studies; D002467:Cell Nucleus / Q000378:metabolism*; D015331:Cohort Studies; D006801:Humans; D034882:Lamins / Q000378:metabolism*; D008565:Membrane Proteins / Q000378:metabolism*; D008875:Middle Aged; D018979:Myositis, Inclusion Body / Q000209:etiology / Q000378:metabolism* / Q000473:pathology*; D009687:Nuclear Proteins / Q000378:metabolism*; D052097:Quadriceps Muscle / Q000378:metabolism* / Q000473:pathology
D034882:Lamins; D008565:Membrane Proteins; D009687:Nuclear Proteins; C092710:emerin