Atrophin proteins: an overview of a new class of nuclear receptor corepressors.
Atrophin proteins: an overview of a new class of nuclear receptor corepressors.
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DOI：
10.1621/nrs.06009
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发表时间：
2008
期刊：
影响因子：
--
通讯作者：
Tsai CC
中科院分区：
文献类型：
--
作者：
Wang L;Tsai CC
多细胞生物的正常发育和生理功能受到复杂的基因转录网络的调控，该网络包括无数的转录因子、与其相关的辅调节因子和多种染色质修饰因子。由这些元件之间的突变引起的异常基因转录调控常常导致发育缺陷和疾病。本文综述了Atrophin家族的蛋白质，包括脊椎动物Atrophin-1（ATN 1），脊椎动物精氨酸-谷氨酸二肽重复蛋白（RERE）和果蝇Atrophin（Atro），我们最近发现的核受体辅阻遏物。Atrophin介导的通路的破坏导致小鼠、斑马鱼和果蝇的多种发育缺陷，而ATN 1的异常形式和RERE的表达水平改变分别与人类的神经退行性疾病和癌症相关。我们在这里提供了一个关于这些Atrophin蛋白的现有知识的概述。我们希望这些关于Atrophin蛋白的信息可能有助于激发新的想法，即这种新发现的核受体辅阻遏物如何帮助特定的核受体和其他转录因子调节基因转录，表现出生理效应并引起疾病。
The normal development and physiological functions of multicellular organisms are regulated by complex gene transcriptional networks that include myriad transcription factors, their associating coregulators, and multiple chromatin-modifying factors. Aberrant gene transcriptional regulation resulting from mutations among these elements often leads to developmental defects and diseases. This review article concentrates on the Atrophin family proteins, including vertebrate Atrophin-1 (ATN1), vertebrate arginine-glutamic acid dipeptide repeats protein (RERE), and Drosophila Atrophin (Atro), which we recently identified as nuclear receptor corepressors. Disruption of Atrophin-mediated pathways causes multiple developmental defects in mouse, zebrafish, and Drosophila, while an aberrant form of ATN1 and altered expression levels of RERE are associated with neurodegenerative disease and cancer in humans, respectively. We here provide an overview of current knowledge about these Atrophin proteins. We hope that this information on Atrophin proteins may help stimulate fresh ideas about how this newly identified class of nuclear receptor corepressors aids specific nuclear receptors and other transcriptional factors in regulating gene transcription, manifesting physiological effects, and causing diseases.