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

FEBS Letters

Kendrick-Jones, J.¹  Morris, G.E.⁴  Simmons, C.⁴  Helliwell, T.R.²  Winder, S.J.¹  Davies, K.E.³ 

[1] MRC Laboratory of Molecular Biology, Hills Road, Cambridge, CB2 2QH, UK;Department of Pathology, University of Liverpool, P.O. Box 147, Liverpool L69 3BX, UK;Molecular Genetics Group, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, OX3 9DU, UK;MRIC Biotechnology Group, N.E. Wales Institute, Deeside, Clwyd CH5 4BR, UK

关键词: Dystrophin;    Muscular dystrophy;    Dermatomyositis;    Monoclonal antibody;    Actin binding;    Neuromuscular junction;   

DOI  :  10.1016/0014-5793(94)01441-3

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

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.

【 授权许可】

Unknown