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首页> 外文期刊>Neuropathology and applied neurobiology >Expression of mutant ubiquitin (UBB(+1)) and p62 in myotilinopathies and desminopathies.
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Expression of mutant ubiquitin (UBB(+1)) and p62 in myotilinopathies and desminopathies.

机译：突变的泛素（UBB（+1））和p62在肌铁蛋白病和脱蛋白病中的表达。

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摘要

Protein aggregates in muscle cells are the morphological hallmark of myofibrillar myopathies, including myotilinopathies and desminopathies. The aim of the present study is to analyse the expression of mutant ubiquitin (UBB(+1)), an aberrant form of ubiquitin which accumulates in certain disorders characterized by intracellular aggregates of proteins, and p62, a multimeric signal protein which plays an active role in aggregate formation, in muscle biopsies from patients suffering from myotilinopathy and desminopathy in order to gain understanding of the mechanisms leading to protein aggregation in these disorders. Single immunohistochemistry, and single- and double-labelling immunofluorescence and confocal microscopy for UBB(+1) and p62, has been performed in muscle biopsies from patients suffering from myotilinopathy and desminopathy. Strong UBB(+1) immunoreactivity, colocalizing with myotilin aggregates, was found in muscle fibres in myotilinopathies. UBB(+1) accumulation, colocalizing with desmin aggregates, also occurs in desminopathies. In addition, strong p62 immunoreactivity colocalizing with myotilin aggregates was observed in myotilinopathies. Similarly, p62 immunoreactivity colocalizing with desmin aggregates was found in desminopathies. The present findings suggest that accumulation of protein aggregates in myotilinopathies and in desminopathies may be related with UBB(+1)/abnormal protein complexes which are resistant to proteasome degradation. Furthermore, these observations suggest a relationship between the presence of p62 and the formation of inclusions in different subtypes of myofibrillar myopathies.
机译：肌肉细胞中的蛋白质聚集体是肌原纤维性肌病的形态标志，包括肌原性肌病和脱髓鞘病。本研究的目的是分析突变泛素（UBB（+1））（一种异常形式的泛素，其在某些疾病中积累，以蛋白质的细胞内聚集体为特征）的表达，以及p62（一种发挥活性的多聚信号蛋白）的表达。在患有肌萎缩性病和脱皮病的患者的肌肉活检中，在聚集物形成中发挥重要作用，以了解导致这些疾病中蛋白质聚集的机制。单免疫组化，单标记和双标记免疫荧光和共聚焦显微镜检查的UBB（+1）和p62，已在肌肉活检中患有肌萎缩和皮下病的患者中进行。强烈的UBB（+1）免疫反应性，与myotilin聚集体共定位，在肌成肌病的肌肉纤维中被发现。与结蛋白聚集体共定位的UBB（+1）积累也发生在结缔组织病中。另外，在肌铁蛋白病中观察到与肌醇蛋白聚集体共定位的强p62免疫反应性。同样，在desminopathies中发现与desmin聚集体共定位的p62免疫反应性。目前的发现表明，在肌铁蛋白病和半脱蛋白病中蛋白质聚集体的积累可能与对蛋白酶体降解具有抗性的UBB（+1）/异常蛋白复合物有关。此外，这些观察结果提示p62的存在与肌原纤维肌病的不同亚型内含物形成之间的关系。

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