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 |
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container_title | Journal of the neurological sciences |
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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.</description><identifier>ISSN: 0022-510X</identifier><identifier>EISSN: 1878-5883</identifier><identifier>DOI: 10.1016/0022-510X(92)90294-U</identifier><identifier>PMID: 1564523</identifier><identifier>CODEN: JNSCAG</identifier><language>eng</language><publisher>Shannon: Elsevier B.V</publisher><subject>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</subject><ispartof>Journal of the neurological sciences, 1992-02, Vol.107 (2), p.233-238</ispartof><rights>1992</rights><rights>1992 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c398t-6245e17275ce7079d9d19e50ce547d2ce391732ddbd99e603b3d7160f5107d353</citedby><cites>FETCH-LOGICAL-c398t-6245e17275ce7079d9d19e50ce547d2ce391732ddbd99e603b3d7160f5107d353</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/0022-510X(92)90294-U$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=5256515$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/1564523$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Augier, Nathalie</creatorcontrib><creatorcontrib>Boucraut, José</creatorcontrib><creatorcontrib>Léger, Jocelyne</creatorcontrib><creatorcontrib>Anoal, Monique</creatorcontrib><creatorcontrib>Nicholson, Louise V.B.</creatorcontrib><creatorcontrib>Voelkel, Marie A.</creatorcontrib><creatorcontrib>Léger, Jean J.</creatorcontrib><creatorcontrib>Pellissier, Jean F.</creatorcontrib><title>A homologue of dystrophin is expressed at the blood vessel membrane of DMD and BMD patients: Immunological evidence</title><title>Journal of the neurological sciences</title><addtitle>J Neurol Sci</addtitle><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.</description><subject>Adult</subject><subject>Antibodies, Monoclonal</subject><subject>Becker's muscular dystrophic</subject><subject>Biological and medical sciences</subject><subject>Blood vessel</subject><subject>blood vessels</subject><subject>Blotting, Western</subject><subject>cell membranes</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Diseases of striated muscles. Neuromuscular diseases</subject><subject>DRP protein</subject><subject>Duchenne's muscular dystrophy</subject><subject>Dystrophin</subject><subject>Dystrophin - analysis</subject><subject>Dystrophin - genetics</subject><subject>Dystrophin-related protein (DRP)</subject><subject>expression</subject><subject>Female</subject><subject>Fluorescent Antibody Technique</subject><subject>Humans</subject><subject>Male</subject><subject>man</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Muscles - pathology</subject><subject>Muscles - physiopathology</subject><subject>Muscular Dystrophies - classification</subject><subject>Muscular Dystrophies - genetics</subject><subject>Muscular Dystrophies - pathology</subject><subject>Neurology</subject><subject>Smooth muscle</subject><subject>X Chromosome</subject><issn>0022-510X</issn><issn>1878-5883</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1992</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkUtv1DAUha0KVKal_wAkLxBqFwE_YjtmgdQXUKmITUfqzkrsG8YoiYOdjOi_r9MZlR1idSXfc46uv4PQG0o-UELlR0IYKwQl96eanWnCdFmsD9CKVqoqRFXxF2j1LHmFjlL6RQiRVaUP0SEVshSMr1A6x5vQhy78nAGHFruHNMUwbvyAfcLwZ4yQEjhcT3jaAG66EBzeLm8d7qFvYj08-a6-X-F6cPgiz7GePAxT-oRv-n4elnBv6w7D1jsYLLxGL9u6S3Cyn8do_eX67vJbcfvj683l-W1hua6mQrJSAFVMCQuKKO20oxoEsSBK5ZgFrqnizLnGaQ2S8IY7RSVp84eV44Ifo_e73DGG3zOkyfQ-Wei6fHOYk1Gs0pT-h5DKklBNZBaWO6GNIaUIrRmj7-v4YCgxSylmIW4W4kYz81SKWWfb233-3PTg_pp2LeT9u_2-ThlUm6Fan55lggkp6HLm550MMrSth2iS9QtQ5yPYybjg_33HI1_4qBs</recordid><startdate>199202</startdate><enddate>199202</enddate><creator>Augier, Nathalie</creator><creator>Boucraut, José</creator><creator>Léger, Jocelyne</creator><creator>Anoal, Monique</creator><creator>Nicholson, Louise V.B.</creator><creator>Voelkel, Marie A.</creator><creator>Léger, Jean J.</creator><creator>Pellissier, Jean F.</creator><general>Elsevier B.V</general><general>Elsevier Science</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>8FD</scope><scope>FR3</scope><scope>M7Z</scope><scope>P64</scope><scope>7X8</scope></search><sort><creationdate>199202</creationdate><title>A homologue of dystrophin is expressed at the blood vessel membrane of DMD and BMD patients: Immunological evidence</title><author>Augier, Nathalie ; Boucraut, José ; Léger, Jocelyne ; Anoal, Monique ; Nicholson, Louise V.B. ; Voelkel, Marie A. ; Léger, Jean J. ; Pellissier, Jean F.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c398t-6245e17275ce7079d9d19e50ce547d2ce391732ddbd99e603b3d7160f5107d353</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1992</creationdate><topic>Adult</topic><topic>Antibodies, Monoclonal</topic><topic>Becker's muscular dystrophic</topic><topic>Biological and medical sciences</topic><topic>Blood vessel</topic><topic>blood vessels</topic><topic>Blotting, Western</topic><topic>cell membranes</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Diseases of striated muscles. Neuromuscular diseases</topic><topic>DRP protein</topic><topic>Duchenne's muscular dystrophy</topic><topic>Dystrophin</topic><topic>Dystrophin - analysis</topic><topic>Dystrophin - genetics</topic><topic>Dystrophin-related protein (DRP)</topic><topic>expression</topic><topic>Female</topic><topic>Fluorescent Antibody Technique</topic><topic>Humans</topic><topic>Male</topic><topic>man</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Muscles - pathology</topic><topic>Muscles - physiopathology</topic><topic>Muscular Dystrophies - classification</topic><topic>Muscular Dystrophies - genetics</topic><topic>Muscular Dystrophies - pathology</topic><topic>Neurology</topic><topic>Smooth muscle</topic><topic>X Chromosome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Augier, Nathalie</creatorcontrib><creatorcontrib>Boucraut, José</creatorcontrib><creatorcontrib>Léger, Jocelyne</creatorcontrib><creatorcontrib>Anoal, Monique</creatorcontrib><creatorcontrib>Nicholson, Louise V.B.</creatorcontrib><creatorcontrib>Voelkel, Marie A.</creatorcontrib><creatorcontrib>Léger, Jean J.</creatorcontrib><creatorcontrib>Pellissier, Jean F.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>Biochemistry Abstracts 1</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of the neurological sciences</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Augier, Nathalie</au><au>Boucraut, José</au><au>Léger, Jocelyne</au><au>Anoal, Monique</au><au>Nicholson, Louise V.B.</au><au>Voelkel, Marie A.</au><au>Léger, Jean J.</au><au>Pellissier, Jean F.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>A homologue of dystrophin is expressed at the blood vessel membrane of DMD and BMD patients: Immunological evidence</atitle><jtitle>Journal of the neurological sciences</jtitle><addtitle>J Neurol Sci</addtitle><date>1992-02</date><risdate>1992</risdate><volume>107</volume><issue>2</issue><spage>233</spage><epage>238</epage><pages>233-238</pages><issn>0022-510X</issn><eissn>1878-5883</eissn><coden>JNSCAG</coden><abstract>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.</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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