Identification of alpha-syntrophin binding to syntrophin triplet, dystrophin, and utrophin.
Identification of alpha-syntrophin binding to syntrophin triplet, dystrophin, and utrophin.
复制标题
鉴定 α-肌营养蛋白与肌营养蛋白三联体、肌营养不良蛋白和肌营养不良蛋白的结合。
DOI：
--
复制
发表时间：
1995
影响因子：
4.8
通讯作者：
K. Campbell
中科院分区：
文献类型：
--
作者：
B. Yang;D. Jung;J. Rafael;J. Chamberlain;K. Campbell
合成营养素代表着肌营养不良蛋白-糖蛋白复合体的三个细胞质成分，该复合体连接着骨骼肌中的细胞骨架和细胞外基质。α-合成营养素现在已经在体外被翻译，并被证明直接与合成营养素三联体的所有三个成分和营养不良蛋白相关联。体外翻译的71 kDa非肌营养不良蛋白亚型，含有富含半胱氨酸半胱氨酸/C末端结构域，也可以与合体滋养蛋白三联体相互作用。通过比较α-合成素与7个重叠的人抗肌营养不良蛋白融合蛋白的相互作用，将该融合蛋白结合基序定位于外显子73和74，含有3447-3481个氨基酸。在这个结合基序中可能存在多个合并素相互作用位点。α-合成营养素还可以直接与C-末端的促性腺激素融合蛋白相互作用。在对照小鼠肌肉中，α-合成素定位于肌肉肌膜和神经肌肉接头。然而，与中营养素类似，α-合成素仅存在于MDX小鼠肌肉中缺乏dystrophin的神经肌肉接头处。我们的数据表明，α-合成素结合了所有的合成素亚型，并且合成素通过包括3447-3481位氨基酸在内的Ddystrophin外显子73和74上的多个结合位点直接与dystrophin相互作用。
Syntrophin represents three cytoplasmic components of the dystrophin-glycoprotein complex that links the cytoskeleton to the extracellular matrix in skeletal muscle. alpha-Syntrophin has now been translated in vitro and shown to associate directly with all three components of the syntrophin triplet and with dystrophin. The in vitro translated 71-kDa non-muscle dystrophin isoform, containing the cystein-rich/C-terminal domain, can also interact with the syntrophin triplet. The syntrophin binding motif in dystrophin was localized to exons 73 and 74 including amino acids 3447-3481 by comparing the interactions of alpha-syntrophin and seven overlapping human dystrophin fusion proteins. More than one syntrophin interaction site in this binding motif was suggested. alpha-Syntrophin also interacts directly with a C-terminal utrophin fusion protein. alpha-Syntrophin is localized to the muscle sarcolemma as well as to the neuromuscular junction in control mouse muscle. However, similar to utrophin, alpha-syntrophin is only present at the neuromuscular junction in mdx mouse muscle in which dystrophin is absent. Our data suggest that alpha-syntrophin binds all syntrophin isoforms, and syntrophin directly interacts with dystrophin through more than one binding site in dystrophin exons 73 and 74 including amino acids 3447-3481.