Zfrp8 forms a complex with fragile-X mental retardation protein and regulates its localization and function.
Zfrp8 forms a complex with fragile-X mental retardation protein and regulates its localization and function.
复制标题
DOI：
10.1016/j.ydbio.2015.12.008
复制
发表时间：
2016-02-15
影响因子：
2.7
通讯作者：
Steward R
中科院分区：
文献类型：
--
作者：
Tan W;Schauder C;Naryshkina T;Minakhina S;Steward R
脆性X综合征是自闭症和精神残疾最常见的遗传原因。Fmr 1（脆性X智力迟钝1）基因在人类和果蝇中对于维持神经干细胞是必不可少的，并且Fmr 1缺失会导致人类和果蝇的神经和生殖发育缺陷。FMRP（Fragile-X Mental Retardation Protein）是一种核质穿梭蛋白，参与mRNA沉默和翻译抑制。Zfrp 8和Fmr 1在果蝇卵巢中具有重要的功能。在这项研究中，我们确定了FMRP，Nufip（核脆性X智力迟钝蛋白相互作用蛋白）和Tral（Trailer Hitch）作为Zfrp 8蛋白复合物的组成部分。我们表明，Zfrp 8是必需的细胞核中，并控制FMRP在细胞质中的定位。此外，我们证明，Zfrp 8基因相互作用与Fmr 1和tral的拮抗方式。Zfrp 8和FMRP都以相反的方式控制异染色质包装。我们认为Zfrp 8作为一种伴侣蛋白，控制参与细胞核中RNA加工的蛋白质复合物。
Fragile-X syndrome is the most commonly inherited cause of autism and mental disabilities. The Fmr1 (Fragile-X Mental Retardation 1) gene is essential in humans and Drosophila for the maintenance of neural stem cells, and Fmr1 loss results in neurological and reproductive developmental defects in humans and flies. FMRP (Fragile-X Mental Retardation Protein) is a nucleo-cytoplasmic shuttling protein, involved in mRNA silencing and translational repression. Both Zfrp8 and Fmr1 have essential functions in the Drosophila ovary. In this study, we identified FMRP, Nufip (Nuclear Fragile-X Mental Retardation Protein-interacting Protein) and Tral (Trailer Hitch) as components of a Zfrp8 protein complex. We show that Zfrp8 is required in the nucleus, and controls localization of FMRP in the cytoplasm. In addition, we demonstrate that Zfrp8 genetically interacts with Fmr1 and tral in an antagonistic manner. Zfrp8 and FMRP both control heterochromatin packaging, also in opposite ways. We propose that Zfrp8 functions as a chaperone, controlling protein complexes involved in RNA processing in the nucleus.
影响因子：
--
作者：
Bauer CR;Epstein AM;Sweeney SJ;Zarnescu DC;Bosco G
通讯作者：
Bosco G