A homologue of dystrophin is expressed at the blood vessel membrane of DMD and BMD patients: Immunological evidence

Muscles from Becker muscular dystrophy (BMD) and Duchenne muscular dystrophy (DMD) patients were analysed using monoclonal and polyclonal antibodies raised against different regions of the dystrophin molecule. On blot, two of the antibodies detected a protein of Mr 400K in muscle extracts from all p...

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Veröffentlicht in:Journal of the neurological sciences 1992-02, Vol.107 (2), p.233-238
Hauptverfasser: Augier, Nathalie, Boucraut, José, Léger, Jocelyne, Anoal, Monique, Nicholson, Louise V.B., Voelkel, Marie A., Léger, Jean J., Pellissier, Jean F.
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container_end_page 238
container_issue 2
container_start_page 233
container_title Journal of the neurological sciences
container_volume 107
creator Augier, Nathalie
Boucraut, José
Léger, Jocelyne
Anoal, Monique
Nicholson, Louise V.B.
Voelkel, Marie A.
Léger, Jean J.
Pellissier, Jean F.
description Muscles from Becker muscular dystrophy (BMD) and Duchenne muscular dystrophy (DMD) patients were analysed using monoclonal and polyclonal antibodies raised against different regions of the dystrophin molecule. On blot, two of the antibodies detected a protein of Mr 400K in muscle extracts from all patients, including a BMD patient with a deletion which spanned more than 40% of the central rod domain of the Xp21 encoded dystrophin. Immunocytochemical labelling of tissue sections from the same patients showed that the same two antibodies labelled a protein at the surface membrane of smooth muscle fibers in blood vessels of both BMD and DMD muscles. Thus we have demonstrated a 400K blood vessel-associated protein, which is immunologically homologous with dystrophin, for at least two epitopes from the carboxy terminal and the central rod domains must be encoded by another gene than the dystrophin gene.
doi_str_mv 10.1016/0022-510X(92)90294-U
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On blot, two of the antibodies detected a protein of Mr 400K in muscle extracts from all patients, including a BMD patient with a deletion which spanned more than 40% of the central rod domain of the Xp21 encoded dystrophin. Immunocytochemical labelling of tissue sections from the same patients showed that the same two antibodies labelled a protein at the surface membrane of smooth muscle fibers in blood vessels of both BMD and DMD muscles. Thus we have demonstrated a 400K blood vessel-associated protein, which is immunologically homologous with dystrophin, for at least two epitopes from the carboxy terminal and the central rod domains must be encoded by another gene than the dystrophin gene.</abstract><cop>Shannon</cop><pub>Elsevier B.V</pub><pmid>1564523</pmid><doi>10.1016/0022-510X(92)90294-U</doi><tpages>6</tpages></addata></record>
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subjects Adult
Antibodies, Monoclonal
Becker's muscular dystrophic
Biological and medical sciences
Blood vessel
blood vessels
Blotting, Western
cell membranes
Child
Child, Preschool
Diseases of striated muscles. Neuromuscular diseases
DRP protein
Duchenne's muscular dystrophy
Dystrophin
Dystrophin - analysis
Dystrophin - genetics
Dystrophin-related protein (DRP)
expression
Female
Fluorescent Antibody Technique
Humans
Male
man
Medical sciences
Middle Aged
Muscles - pathology
Muscles - physiopathology
Muscular Dystrophies - classification
Muscular Dystrophies - genetics
Muscular Dystrophies - pathology
Neurology
Smooth muscle
X Chromosome
title A homologue of dystrophin is expressed at the blood vessel membrane of DMD and BMD patients: Immunological evidence
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