散发性包涵体肌炎（sIBM）患者肌肉细胞核中核纤层蛋白和emerin的分布改变。
Altered distribution of lamin and emerin in muscle nuclei of sIBM patients.
作者信息
Fidzianska A, Glinka Z, Kaminska A, Niebroj-Dobosz I
机构信息
Neuromuscular Unit, Medical Research Center, Polish Academy of Sciences, Pawinskiego 5 str., 02-106 Warsaw, Poland.
出版信息
Clin Neuropathol. 2008 Nov-Dec;27(6):424-9. doi: 10.5414/npp27424.
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.
目的
散发性包涵体肌炎（sIBM）是一种慢性获得性炎性肌病。sIBM的病因尚不清楚，其发病机制存在争议。有一种假说[卡尔帕蒂和卡彭特1993年]认为，镶边空泡是由核崩解产生的，而IBM细丝是由核基质的成分形成的。
材料与方法
为检测核膜蛋白，采用免疫组织化学和免疫化学技术对6例IBM患者进行了研究。
结果
结果表明，在10%-15%的肌核内部，emerin和核纤层蛋白A/C包涵体持续出现。这一发现表明核纤层蛋白A/C表位定位异常，在受影响的细胞核中存在推测的核纤层蛋白A（67KDu）和emerin。
结论
我们支持这样的观点，即截短的、改变的核纤层蛋白A参与了核崩解和镶边空泡的形成。
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