Novel cytoplasmic immunolocalization of RNA polymerase II in inclusion-body myositis muscle.
Novel cytoplasmic immunolocalization of RNA polymerase II in inclusion-body myositis muscle.
复制标题
RNA 聚合酶 II 在包涵体肌炎肌肉中的新型细胞质免疫定位。
DOI：
10.1097/00001756-200107030-00010
复制
发表时间：
2001
期刊：
影响因子：
1.7
通讯作者：
Askanas,V
中科院分区：
文献类型：
--
作者：
Wilczynski,GM;Engel,WK;Askanas,V
散发性包涵体肌炎(IBM)是一种老年人进行性退行性肌肉疾病。最近，基因表达和RNA代谢的异常被认为与IBM的致病级联有关。现在，我们使用特征良好的表位特异性抗体证明，RNA聚合酶II的最大亚单位异常地聚集在IBM肌肉纤维的细胞质中，在那里它与IBM成对螺旋细丝上的磷酸化tau共同定位。由于RNA聚合酶II是参与转录和mRNA加工的关键核因子，我们的结果支持这样的假设，即这两个过程中的一个或两个过程的异常可能部分是由RNA聚合酶II的病理性转运引起的，而异常转运可能是IBM致病级联反应中的一个重要因素。
Sporadic inclusion-body myositis (IBM) is a progressive degenerative muscle disease of older persons. Abnormalities of gene-expression and RNA metabolism have recently been proposed to contribute to the IBM pathogenic cascade. We now demonstrate, using well characterized, epitope-specific antibodies, that the largest subunit of RNA polymerase II is abnormally accumulated in the cytoplasm of IBM muscle fibers, where it is co-localized with phosphorylated tau on IBM paired helical filaments. Since RNA polymerase II is a crucial nuclear factor involved in both transcription and mRNA processing, our results support the hypothesis that abnormality of either or both of those processes might be caused, in part, by pathological trafficking of RNA polymerase II, and that abnormal trafficking might be an important factor in the IBM pathogenic cascade.