Altered distribution of lamin and emerin in muscle nuclei of sIBM patients

• Published in: Clinical neuropathology Vol. 27; no. 6; p. 424
• Main Authors: Fidzianska, A, Glinka, Z, Kaminska, A, Niebroj-Dobosz, I
• Format: Journal Article
• Language: English
• Published: Germany 01.11.2008
• Subjects: Aged; Case-Control Studies; Cell Nucleus - metabolism; Cohort Studies; Humans; Lamins - metabolism; Membrane Proteins - metabolism; Middle Aged; Myositis, Inclusion Body - etiology; Myositis, Inclusion Body - metabolism; Myositis, Inclusion Body - pathology; Nuclear Proteins - metabolism; Quadriceps Muscle - metabolism; Quadriceps Muscle - pathology
• ISSN: 0722-5091
• DOI: 10.5414/npp27424

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. For nuclear membrane protein detection, six IBM patients were studied using immunohistochemical and immunochemical techniques. 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. We support the suggestion that truncated, changed lamin A protein takes part in nuclear disintegration and rimmed vacuole formation.