Yeadon J E, Lin H, Dyer S M, Burden S J
Department of Biology, Massachusetts Institute of Technology, Cambridge 02139.
J Cell Biol. 1991 Nov;115(4):1069-76. doi: 10.1083/jcb.115.4.1069.
A subsynaptic protein of Mr approximately 300 kD is a major component of Torpedo electric organ postsynaptic membranes and copurifies with the AChR and the 43-kD subsynaptic protein. mAbs against this protein react with neuromuscular synapses in higher vertebrates, but not at synapses in dystrophic muscle. The Torpedo 300-kD protein comigrates in SDS-PAGE with murine dystrophin and reacts with antibodies against murine dystrophin. The sequence of a partial cDNA isolated by screening an expression library with mAbs against the Torpedo 300-kD protein shows striking homology to mammalian dystrophin, and in particular to the b isoform of dystrophin. These results indicate that dystrophin is a component of the postsynaptic membrane at neuromuscular synapses and raise the possibility that loss of dystrophin from synapses in dystrophic muscle may have consequences that contribute to muscular dystrophy.
一种分子量约为300 kD的突触后蛋白是电鳐电器官突触后膜的主要成分,与乙酰胆碱受体(AChR)和43-kD突触后蛋白共同纯化。针对该蛋白的单克隆抗体可与高等脊椎动物的神经肌肉突触发生反应,但在营养不良性肌肉的突触处则无反应。电鳐的300-kD蛋白在SDS-PAGE中与小鼠肌营养不良蛋白迁移率相同,并与抗小鼠肌营养不良蛋白的抗体发生反应。通过用抗电鳐300-kD蛋白的单克隆抗体筛选表达文库分离得到的部分cDNA序列与哺乳动物肌营养不良蛋白具有显著同源性,尤其是与肌营养不良蛋白的b亚型。这些结果表明,肌营养不良蛋白是神经肌肉突触后膜的一个组成部分,并增加了以下可能性:营养不良性肌肉突触中肌营养不良蛋白的缺失可能产生导致肌肉营养不良的后果。