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Tissue specificity in the nuclear envelope supports its functional complexity

机译：核膜中的组织特异性支持其功能复杂性

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Nuclear envelope links to inherited disease gave the conundrum of how mutations in near-ubiquitous proteins can yield many distinct pathologies, each focused in different tissues. One conundrum-resolving hypothesis is that tissue-specific partner proteins mediate these pathologies. Such partner proteins may have now been identified with recent proteome studies determining nuclear envelope composition in different tissues. These studies revealed that the majority of the total nuclear envelope proteins are tissue restricted in their expression. Moreover, functions have been found for a number these tissue-restricted nuclear envelope proteins that fit with mechanisms proposed to explain how the nuclear envelope could mediate disease, including defects in mechanical stability, cell cycle regulation, signaling, genome organization, gene expression, nucleocytoplasmic transport, and differentiation. The wide range of functions to which these proteins contribute is consistent with not only their involvement in tissue-specific nuclear envelope disease pathologies, but also tissue evolution.
机译：核被膜与遗传性疾病的联系给人们带来了难题，即普遍存在的蛋白质中的突变如何产生许多不同的病理学，每个病理学都集中在不同的组织上。解决难题的一种假设是组织特异性伴侣蛋白介导了这些病理。这类伴侣蛋白现在可能已经通过最近的蛋白质组研究确定了不同组织中的核包膜成分。这些研究表明，总核包膜蛋白中的大多数在表达上受到组织的限制。此外，已经发现了许多这些组织限制性的核被膜蛋白的功能，这些蛋白与为解释核被膜如何介导疾病而提出的机制相适应，包括机械稳定性，细胞周期调控，信号传导，基因组组织，基因表达，核质的缺陷。运输和差异化。这些蛋白质所发挥的广泛功能不仅与其在组织特异性核被膜疾病病理学中的参与，而且还与组织进化相一致。

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