Todd Peter K, Malter James S
Department of Pathology, Medical Scientist and Neuroscience Training Program, University of Wisconsin, 600 Highland Drive, Madison, WI 53792, USA.
J Neurosci Res. 2002 Dec 1;70(5):623-30. doi: 10.1002/jnr.10453.
Fragile X syndrome is the most common cause of mental retardation known to be inherited. The syndrome results from the suppressed expression of a single protein, the fragile X mental retardation protein (FMRP). Understanding the function and regulation of FMRP can, therefore, offer insights into both the pathophysiology of fragile X syndrome and the molecular mechanisms of learning and memory. We provide an overview of current concepts of how FMRP functions in the nervous system, with special emphasis on recent evidence that FMRP has a role in metabotropic glutamate receptor-activated protein translation and synaptic plasticity.
脆性X综合征是已知的遗传性智力障碍最常见的病因。该综合征是由一种单一蛋白质——脆性X智力障碍蛋白(FMRP)的表达受抑制所致。因此,了解FMRP的功能和调控有助于深入了解脆性X综合征的病理生理学以及学习和记忆的分子机制。我们概述了FMRP在神经系统中发挥作用的当前概念,特别强调了最近的证据,即FMRP在代谢型谷氨酸受体激活的蛋白翻译和突触可塑性中起作用。