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Full-length and short forms of utrophin, the dystrophin-related protein

Authors :
Timothy R. Helliwell
John Kendrick-Jones
Kay E. Davies
C. Simmons
Nguyen thi Man
Glenn E. Morris
Steven J. Winder
Source :
FEBS letters. 358(3)
Publication Year :
1995

Abstract

All previous studies of the localization of utrophin (the dystrophin-related protein) in muscle and other tissues have been performed only with antibodies against the C-terminal region of the protein. Since several short forms of dystrophin, the apodystrophins, are produced from the 3′ end of the dystrophin gene, there is a possibility that similar short forms of utrophin exist and that these could be responsible for some of the many different localizations of ‘utrophin’ in muscle. We have produced a new panel of 15 mAbs against the N-terminal region of utrophin and we have used it together with mAbs against the C-terminal region to show that full-length utrophin is present at neuromuscular junctions, in nerves, blood vessels and capillaries in normal muscle and in the sarcolemma of patients with muscular dystrophy and dermatomyositis. However, two of the 15 mAbs also recognised rat/mouse utrophin and both of these detected an additional 62 kDa protein on Western blots of rat C6 glioma cells. This potential 62 kDa ‘apo-utrophin’ was not detected in human cerebral cortex, in rat Schwannoma cells nor in any of the non-nerve cells and tissues tested.

Details

ISSN :
00145793
Volume :
358
Issue :
3
Database :
OpenAIRE
Journal :
FEBS letters
Accession number :
edsair.doi.dedup.....f8ba3a693dab1b56eb37b32951c3ae93