Kramarcy N R, Vidal A, Froehner S C, Sealock R
Department of Physiology, University of North Carolina, Chapel Hill 27599.
J Biol Chem. 1994 Jan 28;269(4):2870-6.
Electric tissue syntrophin, originally described as an M(r) 58,000 postsynaptic protein having homologs in mammalian muscle, was previously shown to associate with dystrophin in Triton extracts of Torpedo postsynaptic membranes. It also associates with the Torpedo M(r) 87,000 postsynaptic protein (87K), the core of which is a superdomain homologous to the cysteine-rich (CR) and COOH-terminal (CT) domains of human dystrophin. Using immunoaffinity purifications from various rat tissues and immunoblotting, we find that syntrophin associates with dystrophin, utrophin (the chromosome 6-encoded dystrophin homolog formerly known as dystrophin-related protein), multiple proteins which are cross-reactive with 87K, and two subfamilies of 71K-like proteins (CRCT-containing proteins encoded by the dystrophin gene under the control of an alternative promoter in intron 62). One 71K subfamily retains the dystrophin COOH-terminal sequence; the other has an alternative COOH-terminal sequence caused by deletion of the penultimate exon by alternative splicing. The relative masses of the members of the subfamilies suggest they arise by alternative splicing at other previously described sites within CT. These results establish that syntrophin is a general ligand for the CRCT domain in mammalian dystrophin and its homologs. They also reveal a greater diversity in 71K proteins than has previously been apparent.
电组织肌养蛋白最初被描述为一种分子量为58,000的突触后蛋白,在哺乳动物肌肉中有同源物,先前已证明它在电鳐突触后膜的Triton提取物中与肌营养不良蛋白相关联。它还与电鳐分子量为87,000的突触后蛋白(87K)相关联,其核心是一个与人类肌营养不良蛋白的富含半胱氨酸(CR)和羧基末端(CT)结构域同源的超结构域。通过从各种大鼠组织中进行免疫亲和纯化和免疫印迹分析,我们发现肌养蛋白与肌营养不良蛋白、抗肌萎缩蛋白(6号染色体编码的肌营养不良蛋白同源物,以前称为肌营养不良蛋白相关蛋白)、多种与87K交叉反应的蛋白以及两个71K样蛋白亚家族(由肌营养不良蛋白基因在第62内含子中的一个替代启动子控制下编码的含CRCT蛋白)相关联。一个71K亚家族保留了肌营养不良蛋白的羧基末端序列;另一个具有因通过替代剪接缺失倒数第二个外显子而产生的替代羧基末端序列。亚家族成员的相对分子量表明它们是通过在CT内其他先前描述的位点进行替代剪接产生的。这些结果表明,肌养蛋白是哺乳动物肌营养不良蛋白及其同源物中CRCT结构域的通用配体。它们还揭示了71K蛋白的多样性比以前明显更大。