Localization of the DMDL gene-encoded dystrophin-related protein using a panel of nineteen monoclonal antibodies: presence at neuromuscular junctions, in the sarcolemma of dystrophic skeletal muscle, in vascular and other smooth muscles, and in proliferating brain cell lines.
Localization of the DMDL gene-encoded dystrophin-related protein using a panel of nineteen monoclonal antibodies: presence at neuromuscular junctions, in the sarcolemma of dystrophic skeletal muscle, in vascular and other smooth muscles, and in proliferating brain cell lines.
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DOI：
10.1083/jcb.115.6.1695
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发表时间：
1991-12
期刊：
影响因子：
--
通讯作者：
Morris GE
中科院分区：
文献类型：
--
作者：
Nguyen TM;Ellis JM;Love DR;Davies KE;Gatter KC;Dickson G;Morris GE
针对DMDL基因蛋白产物上不同表位的单抗已经被提出，DMDL基因是Dstrophin的X连锁DMD基因的常染色体同源物。这些抗体提供了直接证据，表明DMDL蛋白位于正常和MDX小鼠肋间肌神经肌肉连接的乙酰胆碱受体附近。在骨骼肌以外的组织中，主要的位置是平滑肌，特别是在血管系统中，这可能是先前通过Western blotting证明的广泛组织分布的原因。DMDL蛋白在正常肌肉的非交界性肌膜中未检测到，但在Duchenne肌营养不良症患者的非交界性肌膜中可观察到，Duchenne肌营养不良症患者的非交界性肌膜中Dstrophin本身缺失或显著减少。然而，DMDL蛋白的表达并不局限于平滑肌和骨骼肌，因为在胶质细胞和雪旺细胞来源的转化脑细胞系中都存在相对大量的DMDL蛋白。这与成人脑组织中低水平的DMDL蛋白形成了鲜明对比。
mAbs have been raised against different epitopes on the protein product of the DMDL gene, which is an autosomal homologue of the X-linked DMD gene for dystrophin. These antibodies provide direct evidence that DMDL protein is localized near acetylcholine receptors at neuromuscular junctions in normal and mdx mouse intercostal muscle. The primary location in tissues other than skeletal muscle is smooth muscle, especially in the vascular system, which may account for the wide tissue distribution previously demonstrated by Western blotting. The DMDL protein was undetectable in the nonjunctional sarcolemma of normal human muscle, but was observed in nonjunctional sarcolemma of Duchenne muscular dystrophy patients, where dystrophin itself is absent or greatly reduced. The expression of DMDL protein is not restricted to smooth and skeletal muscle, however, since relatively large amounts are present in transformed brain cell lines of both glial and Schwann cell origin. This contrasts with the low levels of DMDL protein in adult brain tissue.