β-dystrobrevin, a member of the dystrophin-related protein family
β-dystrobrevin, a member of the dystrophin-related protein family
复制标题
DOI：
10.1073/pnas.95.1.241
复制
发表时间：
1998-01-06
影响因子：
11.1
通讯作者：
Davies, KE
中科院分区：
文献类型：
--
作者：
Blake, DJ;Nawrotzki, R;Davies, KE
抗肌萎缩蛋白及其相关蛋白在正常肌肉功能中的重要性现已得到充分证实。这些蛋白质中的许多在非肌肉组织中表达，特别是大脑。在这里，我们描述了β-短肌营养不良蛋白，肌营养不良蛋白相关的蛋白质，是大量表达在大脑和其他组织，但没有发现在肌肉中的表征。与肌营养不良蛋白一样，β-肌营养不良蛋白存在于皮层和海马结构的神经元中，但在脑微血管中未发现。在脑中，β-肌营养不良蛋白与肌营养不良蛋白同种型Dp 71和Dp 140共免疫沉淀。这些数据提供的证据表明，在大脑中的肌营养不良蛋白相关蛋白复合物的组成不同于在肌肉。这一发现可能与影响许多杜氏肌营养不良症患者的认知功能障碍有关。
The importance of dystrophin and its associated proteins in normal muscle function is now well established. Many of these proteins are expressed in nonmuscle tissues, particularly the brain. Here we describe the characterization of beta-dystrobrevin, a dystrophin-related protein that is abundantly expressed in brain and other tissues, but is not found in muscle. beta-dystrobrevin is encoded by a 2.5-kb alternatively spliced transcript that is found throughout the brain, In common with dystrophin, beta-dystrobrevin is found in neurons of the cortex and hippocampal formation but is not found in the brain microvasculature, In the brain, beta-dystrobrevin coimmunoprecipitates with the dystrophin isoforms Dp71 and Dp140. These data provide evidence that the composition of the dystrophin-associated protein complex in the brain differs from that in muscle. This finding may be relevant to the cognitive dysfunction affecting many patients with Duchenne muscular dystrophy.