Anchoring Fibrils and Type VII collagen are Absent From Skin in Severe Recessive Dystrophic Epidermolysis Bullosa
Skin of patients with severe generalized recessive dystrophic epidermolysis bullosa (SGRDEB) was studied by immunoelectron microscopy and immunoblotting with antibodies to type VII collagen, a major structural component of anchoring fibrils. In normal skin, the protein was localized to the dermoepid...
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| Veröffentlicht in: | Journal of investigative dermatology 1989-07, Vol.93 (1), p.3-9 |
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| creator | Bruckner-Tuderman, Leena Mitsuhashi, Yoshihiko Schnyder, Urs W. Bruckner, Peter |
| description | Skin of patients with severe generalized recessive dystrophic epidermolysis bullosa (SGRDEB) was studied by immunoelectron microscopy and immunoblotting with antibodies to type VII collagen, a major structural component of anchoring fibrils. In normal skin, the protein was localized to the dermoepidermal junction zone below the basement membrance and was extractable from the papillary dermis after artifical epidermolysis. In SGRDEB skin, neither immunoreactive material below the basement membrance nor identifiable anchoring fibrils could be recognized and neither the tissue from nor the specific proteolytic fragments of type VII collagen were found in extracts of SGRDEB skin. Very low amounts of type VII collagen α-chains could be detected in cultures of SGRDEB-fibroblasts, whereas normal fibroblasts synthesized more of this collagen. These results suggest that a genetic defect in the correct synthesis, secretion, or in the molecular assembly of type VII collagen may underline SGRDEB. |
| doi_str_mv | 10.1111/1523-1747.ep12277331 |
| format | Article |
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In normal skin, the protein was localized to the dermoepidermal junction zone below the basement membrance and was extractable from the papillary dermis after artifical epidermolysis. In SGRDEB skin, neither immunoreactive material below the basement membrance nor identifiable anchoring fibrils could be recognized and neither the tissue from nor the specific proteolytic fragments of type VII collagen were found in extracts of SGRDEB skin. Very low amounts of type VII collagen α-chains could be detected in cultures of SGRDEB-fibroblasts, whereas normal fibroblasts synthesized more of this collagen. These results suggest that a genetic defect in the correct synthesis, secretion, or in the molecular assembly of type VII collagen may underline SGRDEB.</description><identifier>ISSN: 0022-202X</identifier><identifier>EISSN: 1523-1747</identifier><identifier>DOI: 10.1111/1523-1747.ep12277331</identifier><identifier>PMID: 2746005</identifier><identifier>CODEN: JIDEAE</identifier><language>eng</language><publisher>Danvers, MA: Elsevier Inc</publisher><subject>Adolescent ; Adult ; Biological and medical sciences ; Bullous diseases of the skin ; Collagen - classification ; Collagen - metabolism ; Dermatology ; Epidermolysis Bullosa - genetics ; Epidermolysis Bullosa - metabolism ; Epidermolysis Bullosa - pathology ; Fibroblasts - metabolism ; Fibroblasts - pathology ; Genes, Recessive ; Humans ; Immunohistochemistry ; Medical sciences ; Microscopy, Electron ; Skin - metabolism ; Skin - pathology</subject><ispartof>Journal of investigative dermatology, 1989-07, Vol.93 (1), p.3-9</ispartof><rights>1989 The Society for Investigative Dermatology, Inc</rights><rights>1991 INIST-CNRS</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c504t-bd4eb2613813baef181d126f5069367b3df1de50ff01e00b6371aa09b50946d63</citedby><cites>FETCH-LOGICAL-c504t-bd4eb2613813baef181d126f5069367b3df1de50ff01e00b6371aa09b50946d63</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=19333727$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/2746005$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Bruckner-Tuderman, Leena</creatorcontrib><creatorcontrib>Mitsuhashi, Yoshihiko</creatorcontrib><creatorcontrib>Schnyder, Urs W.</creatorcontrib><creatorcontrib>Bruckner, Peter</creatorcontrib><title>Anchoring Fibrils and Type VII collagen are Absent From Skin in Severe Recessive Dystrophic Epidermolysis Bullosa</title><title>Journal of investigative dermatology</title><addtitle>J Invest Dermatol</addtitle><description>Skin of patients with severe generalized recessive dystrophic epidermolysis bullosa (SGRDEB) was studied by immunoelectron microscopy and immunoblotting with antibodies to type VII collagen, a major structural component of anchoring fibrils. In normal skin, the protein was localized to the dermoepidermal junction zone below the basement membrance and was extractable from the papillary dermis after artifical epidermolysis. In SGRDEB skin, neither immunoreactive material below the basement membrance nor identifiable anchoring fibrils could be recognized and neither the tissue from nor the specific proteolytic fragments of type VII collagen were found in extracts of SGRDEB skin. Very low amounts of type VII collagen α-chains could be detected in cultures of SGRDEB-fibroblasts, whereas normal fibroblasts synthesized more of this collagen. These results suggest that a genetic defect in the correct synthesis, secretion, or in the molecular assembly of type VII collagen may underline SGRDEB.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>Bullous diseases of the skin</subject><subject>Collagen - classification</subject><subject>Collagen - metabolism</subject><subject>Dermatology</subject><subject>Epidermolysis Bullosa - genetics</subject><subject>Epidermolysis Bullosa - metabolism</subject><subject>Epidermolysis Bullosa - pathology</subject><subject>Fibroblasts - metabolism</subject><subject>Fibroblasts - pathology</subject><subject>Genes, Recessive</subject><subject>Humans</subject><subject>Immunohistochemistry</subject><subject>Medical sciences</subject><subject>Microscopy, Electron</subject><subject>Skin - metabolism</subject><subject>Skin - pathology</subject><issn>0022-202X</issn><issn>1523-1747</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1989</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kE-LFDEQxYMo67j6DRRy0Vuv-dPd6b4I47qjAwuCu4q3kE6qd6M9SW-qe2C-vRlmmLlZBAryXj2qfoS85eyK5_rIKyELrkp1BSMXQikp-TOyOH0_JwvGhCgEE79fkleIfxjjdVk1F-RCqLJmrFqQp2WwjzH58EBXvkt-QGqCo_e7Eeiv9ZraOAzmAQI1CeiyQwgTXaW4oXd_faD53cEWsvQDLCD6LdAvO5xSHB-9pTejd5A2cdihR_p5HoaI5jV50ZsB4c2xX5Kfq5v762_F7fev6-vlbWErVk5F50roRM1lw2VnoOcNd1zUfcXqVtaqk67nDirW94wDY10tFTeGtV3F2rJ2tbwkHw65Y4pPM-CkNx4t5HMCxBm1alkrq6bJxvJgtCkiJuj1mPzGpJ3mTO9J6z1SvUeqz6Tz2Ltj_txtwJ2Gjmiz_v6oG7Rm6JMJ1uM5u5VSKqHOOcFMc4KTocq3i6bM-qeDDhnW1kPSaD0EC84nsJN20f9_0X-VgqRG</recordid><startdate>19890701</startdate><enddate>19890701</enddate><creator>Bruckner-Tuderman, Leena</creator><creator>Mitsuhashi, Yoshihiko</creator><creator>Schnyder, Urs W.</creator><creator>Bruckner, Peter</creator><general>Elsevier Inc</general><general>Nature Publishing</general><scope>6I.</scope><scope>AAFTH</scope><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>19890701</creationdate><title>Anchoring Fibrils and Type VII collagen are Absent From Skin in Severe Recessive Dystrophic Epidermolysis Bullosa</title><author>Bruckner-Tuderman, Leena ; Mitsuhashi, Yoshihiko ; Schnyder, Urs W. ; Bruckner, Peter</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c504t-bd4eb2613813baef181d126f5069367b3df1de50ff01e00b6371aa09b50946d63</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1989</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Biological and medical sciences</topic><topic>Bullous diseases of the skin</topic><topic>Collagen - classification</topic><topic>Collagen - metabolism</topic><topic>Dermatology</topic><topic>Epidermolysis Bullosa - genetics</topic><topic>Epidermolysis Bullosa - metabolism</topic><topic>Epidermolysis Bullosa - pathology</topic><topic>Fibroblasts - metabolism</topic><topic>Fibroblasts - pathology</topic><topic>Genes, Recessive</topic><topic>Humans</topic><topic>Immunohistochemistry</topic><topic>Medical sciences</topic><topic>Microscopy, Electron</topic><topic>Skin - metabolism</topic><topic>Skin - pathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Bruckner-Tuderman, Leena</creatorcontrib><creatorcontrib>Mitsuhashi, Yoshihiko</creatorcontrib><creatorcontrib>Schnyder, Urs W.</creatorcontrib><creatorcontrib>Bruckner, Peter</creatorcontrib><collection>ScienceDirect Open Access Titles</collection><collection>Elsevier:ScienceDirect:Open Access</collection><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>Journal of investigative dermatology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Bruckner-Tuderman, Leena</au><au>Mitsuhashi, Yoshihiko</au><au>Schnyder, Urs W.</au><au>Bruckner, Peter</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Anchoring Fibrils and Type VII collagen are Absent From Skin in Severe Recessive Dystrophic Epidermolysis Bullosa</atitle><jtitle>Journal of investigative dermatology</jtitle><addtitle>J Invest Dermatol</addtitle><date>1989-07-01</date><risdate>1989</risdate><volume>93</volume><issue>1</issue><spage>3</spage><epage>9</epage><pages>3-9</pages><issn>0022-202X</issn><eissn>1523-1747</eissn><coden>JIDEAE</coden><abstract>Skin of patients with severe generalized recessive dystrophic epidermolysis bullosa (SGRDEB) was studied by immunoelectron microscopy and immunoblotting with antibodies to type VII collagen, a major structural component of anchoring fibrils. In normal skin, the protein was localized to the dermoepidermal junction zone below the basement membrance and was extractable from the papillary dermis after artifical epidermolysis. In SGRDEB skin, neither immunoreactive material below the basement membrance nor identifiable anchoring fibrils could be recognized and neither the tissue from nor the specific proteolytic fragments of type VII collagen were found in extracts of SGRDEB skin. Very low amounts of type VII collagen α-chains could be detected in cultures of SGRDEB-fibroblasts, whereas normal fibroblasts synthesized more of this collagen. These results suggest that a genetic defect in the correct synthesis, secretion, or in the molecular assembly of type VII collagen may underline SGRDEB.</abstract><cop>Danvers, MA</cop><pub>Elsevier Inc</pub><pmid>2746005</pmid><doi>10.1111/1523-1747.ep12277331</doi><tpages>7</tpages><oa>free_for_read</oa></addata></record> |
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| language | eng |
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| source | MEDLINE; EZB-FREE-00999 freely available EZB journals; Alma/SFX Local Collection |
| subjects | Adolescent Adult Biological and medical sciences Bullous diseases of the skin Collagen - classification Collagen - metabolism Dermatology Epidermolysis Bullosa - genetics Epidermolysis Bullosa - metabolism Epidermolysis Bullosa - pathology Fibroblasts - metabolism Fibroblasts - pathology Genes, Recessive Humans Immunohistochemistry Medical sciences Microscopy, Electron Skin - metabolism Skin - pathology |
| title | Anchoring Fibrils and Type VII collagen are Absent From Skin in Severe Recessive Dystrophic Epidermolysis Bullosa |
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