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XB-ART-56739
J Cell Biol 1991 Dec 01;1156:1695-700. doi: 10.1083/jcb.115.6.1695.
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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.

Nguyen TM , Ellis JM , Love DR , Davies KE , Gatter KC , Dickson G , Morris GE .


Abstract
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.

PubMed ID: 1757469
PMC ID: PMC2289198
Article link: J Cell Biol


Species referenced: Xenopus
Genes referenced: dmd dmd.2
Antibodies: DMD Ab1 DMD Ab2 DMD Ab3 UTRN Ab1 Utrn Ab2

References [+] :
Bar, A novel product of the Duchenne muscular dystrophy gene which greatly differs from the known isoforms in its structure and tissue distribution. 1990, Pubmed