A homologue of dystrophin is expressed at the neuromuscular junctions of normal individuals and DMD patients, and of normal and mdx mice Immunological evidence

FEBS Letters

Tome, F.M.S.³  Robert, A.⁵  Mornet, D.⁵  Fardeau, M.³  Léger, J.J.⁵  Léger, J.O.C.⁵  Nicholson, L.V.B.²  Voit, T.¹  Augier, N.⁵  Pons, F.⁴ 

[1] Universitäts-Kinderklinik, Moorenstr. 5, D-4000 Düsseldorf, Germany;Muscular Dystrophy Group Research Laboratories, Regional Neurological Centre, New-Castle General Hospital, Newcastle upon Tyne NE4 6BE, UK;Développement, Pathologie, Régénération du Système Neuromusculaire, INSERM U153, 17 rue du Fer à Moulin, 75005 Paris, France;Pathologie Générale, Faculté de Médecine, Place Henri IV, 34000 Montpellier, France;Pathologie Moléculaire du Muscle, INSERM U300, Faculté de Pharmacie, Av. Ch. Flahaut, 34060 Montpellier cedex 1, France

关键词: Dystrophin;    Neuromuscular junction (NMJ);    Dystrophin related protein (DRP);   

DOI  :  10.1016/0014-5793(91)80468-I

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

Polyclonal and monoclonal antibodies, which recognize different regions and epitopes of the dystrophin molecule, bind to a protein of M _r 400 000 which is present in extracts of mdx muscle from regions which contain neuromuscular junctions (NMJ) and is absent from those which do not. This NMJ-associated homologue of dystrophin has at least 2 epitopes which are different to the usual Xp21 form of dystrophin expressed along the sarcolemma of muscle fibres in normal muscles. This protein is also expressed at the NMJ of a DMD patient who lacks the first 52 exons of the Xp21 dystrophin gene and it must therefore be translated from a different gene transcript.

【 授权许可】

Unknown