Altered distribution of lamin and emerin in muscle nuclei of sIBM patients
| Autor: | Anna Fidziańska, Niebroj-Dobosz I, Zofia Glinka, A Kamińska |
|---|---|
| Rok vydání: | 2008 |
| Předmět: |
Emerin
Vacuole Biology Myositis Inclusion Body Quadriceps Muscle Pathology and Forensic Medicine Cohort Studies Pathogenesis Inflammatory myopathy medicine Humans Nuclear membrane Aged Cell Nucleus Rimmed vacuoles Membrane Proteins Nuclear Proteins General Medicine Middle Aged Nuclear matrix medicine.disease Lamins Cell biology medicine.anatomical_structure Neurology Biochemistry Case-Control Studies Neurology (clinical) Lamin |
| Zdroj: | Clinical Neuropathology. 27:424-429 |
| ISSN: | 0722-5091 |
| DOI: | 10.5414/npp27424 |
| Popis: | 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. Material and 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. Conclusion: We support the suggestion that truncated, changed lamin A protein takes part in nuclear disintegration and rimmed vacuole formation. |
| Databáze: | OpenAIRE |
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