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多系统萎缩小体神经元包涵体。

PML nuclear bodies.

机构信息

INSERM/CNRS/Université Paris Diderot/Institut Universitaire Hématologie U944/ UMR7212, Laboratoire associé de la Ligue Nationale contre le Cancer, Hôpital St. Louis, 1, Av. C. Vellefaux 75475 Paris Cedex 10, France.

出版信息

Cold Spring Harb Perspect Biol. 2010 May;2(5):a000661. doi: 10.1101/cshperspect.a000661. Epub 2010 Apr 21.

Abstract

PML nuclear bodies are matrix-associated domains that recruit an astonishing variety of seemingly unrelated proteins. Since their discovery in the early 1960s, PML bodies have fascinated cell biologists because of their beauty and their tight association with cellular disorders. The identification of PML, a gene involved in an oncogenic chromosomal translocation, as the key organizer of these domains drew instant interest onto them. The multiple levels of PML body regulation by a specific posttranslational modification, sumoylation, have raised several unsolved issues. Functionally, PML bodies may sequester, modify or degrade partner proteins, but in many ways, PML bodies still constitute an enigma.

摘要

PML 核体是与基质相关的结构域,可招募种类繁多的看似不相关的蛋白质。自 20 世纪 60 年代初发现以来,PML 核体因其美丽和与细胞疾病的紧密关联而吸引了细胞生物学家的兴趣。PML 是一种参与致癌染色体易位的基因,被鉴定为这些结构域的关键组织者,这立即引起了人们的关注。PML 体通过特定的翻译后修饰(SUMOylation)进行多层次调节,提出了几个尚未解决的问题。从功能上讲,PML 体可能隔离、修饰或降解伴侣蛋白,但在许多方面,PML 体仍然是一个谜。

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