Basement membrane abnormality in merosin-negative congenital muscular dystrophy
In muscle biopsy specimens from three patients with merosin-negative congenital muscular dystrophy (CMD), there was marked variation in fiber size with evidence of necrotic and regenerating processes and with marked interstitial fibrosis. No muscle fibers or intramuscular nerves stained with merosin...
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| Veröffentlicht in: | Acta neuropathologica 1996, Vol.91 (4), p.332-336 |
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| container_title | Acta neuropathologica |
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| creator | OSARI, S.-I KOBAYASHI, O YAMASHITA, Y MATSUISHI, T GOTO, M TANABE, Y MIGITA, T NONAKA, I |
| description | In muscle biopsy specimens from three patients with merosin-negative congenital muscular dystrophy (CMD), there was marked variation in fiber size with evidence of necrotic and regenerating processes and with marked interstitial fibrosis. No muscle fibers or intramuscular nerves stained with merosin antibody. On electron microscopy, the basement membrane of all the muscle fibers was very poorly discernible and there were occasional disruptions, while the basement membrane of the Schwann cells was well preserved. On the other hand, the sarcolemmal basement membrane in merosin-positive CMD was well preserved even in patient with severe interstitial fibrosis. It remains to be determined how the defective basement membrane in merosin-negative CMD induces defective sarcolemma and eventual fiber necrosis. |
| doi_str_mv | 10.1007/s004010050433 |
| format | Article |
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No muscle fibers or intramuscular nerves stained with merosin antibody. On electron microscopy, the basement membrane of all the muscle fibers was very poorly discernible and there were occasional disruptions, while the basement membrane of the Schwann cells was well preserved. On the other hand, the sarcolemmal basement membrane in merosin-positive CMD was well preserved even in patient with severe interstitial fibrosis. It remains to be determined how the defective basement membrane in merosin-negative CMD induces defective sarcolemma and eventual fiber necrosis.</description><identifier>ISSN: 0001-6322</identifier><identifier>EISSN: 1432-0533</identifier><identifier>DOI: 10.1007/s004010050433</identifier><identifier>PMID: 8928608</identifier><identifier>CODEN: ANPTAL</identifier><language>eng</language><publisher>Berlin: Springer</publisher><subject>Basement Membrane - abnormalities ; Basement Membrane - ultrastructure ; Biological and medical sciences ; Diseases of striated muscles. Neuromuscular diseases ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Medical sciences ; Microscopy, Electron ; Muscle Fibers, Skeletal - pathology ; Muscular Dystrophies - congenital ; Muscular Dystrophies - pathology ; Neurology</subject><ispartof>Acta neuropathologica, 1996, Vol.91 (4), p.332-336</ispartof><rights>1996 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c317t-8ea652e04fe47b7a42522c521499a99025330afd40052e8f603ba17c802633873</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>315,781,785,4025,27927,27928,27929</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=3012611$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/8928608$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>OSARI, S.-I</creatorcontrib><creatorcontrib>KOBAYASHI, O</creatorcontrib><creatorcontrib>YAMASHITA, Y</creatorcontrib><creatorcontrib>MATSUISHI, T</creatorcontrib><creatorcontrib>GOTO, M</creatorcontrib><creatorcontrib>TANABE, Y</creatorcontrib><creatorcontrib>MIGITA, T</creatorcontrib><creatorcontrib>NONAKA, I</creatorcontrib><title>Basement membrane abnormality in merosin-negative congenital muscular dystrophy</title><title>Acta neuropathologica</title><addtitle>Acta Neuropathol</addtitle><description>In muscle biopsy specimens from three patients with merosin-negative congenital muscular dystrophy (CMD), there was marked variation in fiber size with evidence of necrotic and regenerating processes and with marked interstitial fibrosis. No muscle fibers or intramuscular nerves stained with merosin antibody. On electron microscopy, the basement membrane of all the muscle fibers was very poorly discernible and there were occasional disruptions, while the basement membrane of the Schwann cells was well preserved. On the other hand, the sarcolemmal basement membrane in merosin-positive CMD was well preserved even in patient with severe interstitial fibrosis. It remains to be determined how the defective basement membrane in merosin-negative CMD induces defective sarcolemma and eventual fiber necrosis.</description><subject>Basement Membrane - abnormalities</subject><subject>Basement Membrane - ultrastructure</subject><subject>Biological and medical sciences</subject><subject>Diseases of striated muscles. Neuromuscular diseases</subject><subject>Female</subject><subject>Humans</subject><subject>Infant</subject><subject>Infant, Newborn</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Microscopy, Electron</subject><subject>Muscle Fibers, Skeletal - pathology</subject><subject>Muscular Dystrophies - congenital</subject><subject>Muscular Dystrophies - pathology</subject><subject>Neurology</subject><issn>0001-6322</issn><issn>1432-0533</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1996</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpVkE1LxDAQhoMo67p69Cj0IN6qk4826VEXv2BhL3ouaXa6Rpp0TVqh_97ILoKnyWQehnkfQi4p3FIAeRcBBKRXAYLzIzKngrMcCs6PyRwAaF5yxk7JWYyfqWNSFDMyUxVTJag5WT_oiA79kDl0TdAeM934Pjjd2WHKrE__oY_W5x63erDfmJneb9HbQXeZG6MZOx2yzRSH0O8-pnNy0uou4sWhLsj70-Pb8iVfrZ9fl_er3HAqh1yhLguGIFoUspFasIIxUzAqqkpXFbB0P-h2I1IuhqotgTeaSqOAlZwryRfkZr93F_qvEeNQOxsNdl1K0I-xlgqqgjNIYL4HTYoRA7b1Llinw1RTqH8F1v8EJv7qsHhsHG7-6IOxNL8-zHU0umuTMmPjH8aT4pJS_gO4XXcJ</recordid><startdate>1996</startdate><enddate>1996</enddate><creator>OSARI, S.-I</creator><creator>KOBAYASHI, O</creator><creator>YAMASHITA, Y</creator><creator>MATSUISHI, T</creator><creator>GOTO, M</creator><creator>TANABE, Y</creator><creator>MIGITA, T</creator><creator>NONAKA, I</creator><general>Springer</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>7X8</scope></search><sort><creationdate>1996</creationdate><title>Basement membrane abnormality in merosin-negative congenital muscular dystrophy</title><author>OSARI, S.-I ; KOBAYASHI, O ; YAMASHITA, Y ; MATSUISHI, T ; GOTO, M ; TANABE, Y ; MIGITA, T ; NONAKA, I</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c317t-8ea652e04fe47b7a42522c521499a99025330afd40052e8f603ba17c802633873</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1996</creationdate><topic>Basement Membrane - abnormalities</topic><topic>Basement Membrane - ultrastructure</topic><topic>Biological and medical sciences</topic><topic>Diseases of striated muscles. Neuromuscular diseases</topic><topic>Female</topic><topic>Humans</topic><topic>Infant</topic><topic>Infant, Newborn</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Microscopy, Electron</topic><topic>Muscle Fibers, Skeletal - pathology</topic><topic>Muscular Dystrophies - congenital</topic><topic>Muscular Dystrophies - pathology</topic><topic>Neurology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>OSARI, S.-I</creatorcontrib><creatorcontrib>KOBAYASHI, O</creatorcontrib><creatorcontrib>YAMASHITA, Y</creatorcontrib><creatorcontrib>MATSUISHI, T</creatorcontrib><creatorcontrib>GOTO, M</creatorcontrib><creatorcontrib>TANABE, Y</creatorcontrib><creatorcontrib>MIGITA, T</creatorcontrib><creatorcontrib>NONAKA, I</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>MEDLINE - Academic</collection><jtitle>Acta neuropathologica</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>OSARI, S.-I</au><au>KOBAYASHI, O</au><au>YAMASHITA, Y</au><au>MATSUISHI, T</au><au>GOTO, M</au><au>TANABE, Y</au><au>MIGITA, T</au><au>NONAKA, I</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Basement membrane abnormality in merosin-negative congenital muscular dystrophy</atitle><jtitle>Acta neuropathologica</jtitle><addtitle>Acta Neuropathol</addtitle><date>1996</date><risdate>1996</risdate><volume>91</volume><issue>4</issue><spage>332</spage><epage>336</epage><pages>332-336</pages><issn>0001-6322</issn><eissn>1432-0533</eissn><coden>ANPTAL</coden><abstract>In muscle biopsy specimens from three patients with merosin-negative congenital muscular dystrophy (CMD), there was marked variation in fiber size with evidence of necrotic and regenerating processes and with marked interstitial fibrosis. No muscle fibers or intramuscular nerves stained with merosin antibody. On electron microscopy, the basement membrane of all the muscle fibers was very poorly discernible and there were occasional disruptions, while the basement membrane of the Schwann cells was well preserved. On the other hand, the sarcolemmal basement membrane in merosin-positive CMD was well preserved even in patient with severe interstitial fibrosis. It remains to be determined how the defective basement membrane in merosin-negative CMD induces defective sarcolemma and eventual fiber necrosis.</abstract><cop>Berlin</cop><pub>Springer</pub><pmid>8928608</pmid><doi>10.1007/s004010050433</doi><tpages>5</tpages></addata></record> |
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| identifier | ISSN: 0001-6322 |
| ispartof | Acta neuropathologica, 1996, Vol.91 (4), p.332-336 |
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| language | eng |
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| source | MEDLINE; SpringerNature Journals |
| subjects | Basement Membrane - abnormalities Basement Membrane - ultrastructure Biological and medical sciences Diseases of striated muscles. Neuromuscular diseases Female Humans Infant Infant, Newborn Male Medical sciences Microscopy, Electron Muscle Fibers, Skeletal - pathology Muscular Dystrophies - congenital Muscular Dystrophies - pathology Neurology |
| title | Basement membrane abnormality in merosin-negative congenital muscular dystrophy |
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