Gene targeting of BPAG1: Abnormalities in mechanical strength and cell migration in stratified epithelia and neurologic degeneration
BPAG1 is the major antigenic determinant of autoimmune sera of bullous pemphigoid (BP) patients. It is made by stratified squamous epithelia, where it localizes to the inner surface of specialized integrin-mediated adherens junctions (hemidesmosomes). To explore the function of BPAG1 and its relatio...
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| Veröffentlicht in: | Cell 1995-04, Vol.81 (2), p.233-243 |
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| creator | Guo, Lifei Degenstein, Linda Dowling, James Yu, Qian-Chun Wollmann, Robert Perman, Benjamin Fuchs, Elaine |
| description | BPAG1 is the major antigenic determinant of autoimmune sera of bullous pemphigoid (BP) patients. It is made by stratified squamous epithelia, where it localizes to the inner surface of specialized integrin-mediated adherens junctions (hemidesmosomes). To explore the function of BPAG1 and its relation to BP, we targeted the removal of the
BPAG1 gene in mice. Hemidesmosomes are otherwise normal, but they lack the inner plate and have no cytoskeleton attached. Though not affecting cell growth or substratum adhesion, this compromises mechanical integrity and influences migration. Unexpectedly, the mice also develop severe dystonia and sensory nerve degeneration typical of
dystonia musculorum (dt/df) mice. We show that in at least one other strain of
dt/dt mice,
BPAG1 gene is defective. |
| doi_str_mv | 10.1016/0092-8674(95)90333-X |
| format | Article |
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BPAG1 gene in mice. Hemidesmosomes are otherwise normal, but they lack the inner plate and have no cytoskeleton attached. Though not affecting cell growth or substratum adhesion, this compromises mechanical integrity and influences migration. Unexpectedly, the mice also develop severe dystonia and sensory nerve degeneration typical of
dystonia musculorum (dt/df) mice. We show that in at least one other strain of
dt/dt mice,
BPAG1 gene is defective.</description><identifier>ISSN: 0092-8674</identifier><identifier>EISSN: 1097-4172</identifier><identifier>DOI: 10.1016/0092-8674(95)90333-X</identifier><identifier>PMID: 7736575</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Animals ; Autoantigens - genetics ; Autoantigens - isolation & purification ; Carrier Proteins ; Cell Movement ; Collagen ; Collagen Type XVII ; Cytoskeletal Proteins ; Cytoskeleton - pathology ; Cytoskeleton - ultrastructure ; Desmosomes - chemistry ; Desmosomes - pathology ; Desmosomes - ultrastructure ; Dystonia - genetics ; Dystonin ; Epithelium - abnormalities ; Mice ; Mice, Knockout ; Nerve Degeneration - genetics ; Nerve Tissue Proteins ; Non-Fibrillar Collagens ; Pemphigoid, Bullous - genetics ; Phenotype ; RNA, Messenger - analysis ; Sensory Receptor Cells - pathology ; Skin Abnormalities ; Tensile Strength ; Wound Healing - genetics</subject><ispartof>Cell, 1995-04, Vol.81 (2), p.233-243</ispartof><rights>1995</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c520t-e1b0a1c856a264446a08d2b8485b71dfee03c1156f53c581d4e141dd37ed0dc73</citedby><cites>FETCH-LOGICAL-c520t-e1b0a1c856a264446a08d2b8485b71dfee03c1156f53c581d4e141dd37ed0dc73</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/009286749590333X$$EHTML$$P50$$Gelsevier$$Hfree_for_read</linktohtml><link.rule.ids>314,776,780,3537,27901,27902,65306</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/7736575$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Guo, Lifei</creatorcontrib><creatorcontrib>Degenstein, Linda</creatorcontrib><creatorcontrib>Dowling, James</creatorcontrib><creatorcontrib>Yu, Qian-Chun</creatorcontrib><creatorcontrib>Wollmann, Robert</creatorcontrib><creatorcontrib>Perman, Benjamin</creatorcontrib><creatorcontrib>Fuchs, Elaine</creatorcontrib><title>Gene targeting of BPAG1: Abnormalities in mechanical strength and cell migration in stratified epithelia and neurologic degeneration</title><title>Cell</title><addtitle>Cell</addtitle><description>BPAG1 is the major antigenic determinant of autoimmune sera of bullous pemphigoid (BP) patients. It is made by stratified squamous epithelia, where it localizes to the inner surface of specialized integrin-mediated adherens junctions (hemidesmosomes). To explore the function of BPAG1 and its relation to BP, we targeted the removal of the
BPAG1 gene in mice. Hemidesmosomes are otherwise normal, but they lack the inner plate and have no cytoskeleton attached. Though not affecting cell growth or substratum adhesion, this compromises mechanical integrity and influences migration. Unexpectedly, the mice also develop severe dystonia and sensory nerve degeneration typical of
dystonia musculorum (dt/df) mice. We show that in at least one other strain of
dt/dt mice,
BPAG1 gene is defective.</description><subject>Animals</subject><subject>Autoantigens - genetics</subject><subject>Autoantigens - isolation & purification</subject><subject>Carrier Proteins</subject><subject>Cell Movement</subject><subject>Collagen</subject><subject>Collagen Type XVII</subject><subject>Cytoskeletal Proteins</subject><subject>Cytoskeleton - pathology</subject><subject>Cytoskeleton - ultrastructure</subject><subject>Desmosomes - chemistry</subject><subject>Desmosomes - pathology</subject><subject>Desmosomes - ultrastructure</subject><subject>Dystonia - genetics</subject><subject>Dystonin</subject><subject>Epithelium - abnormalities</subject><subject>Mice</subject><subject>Mice, Knockout</subject><subject>Nerve Degeneration - genetics</subject><subject>Nerve Tissue Proteins</subject><subject>Non-Fibrillar Collagens</subject><subject>Pemphigoid, Bullous - genetics</subject><subject>Phenotype</subject><subject>RNA, Messenger - analysis</subject><subject>Sensory Receptor Cells - pathology</subject><subject>Skin Abnormalities</subject><subject>Tensile Strength</subject><subject>Wound Healing - genetics</subject><issn>0092-8674</issn><issn>1097-4172</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1995</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kMFu1DAURS0EKtPCH7SSV4guAnZix0kXSEPVTpEqwQKk7izHfsk8lNhT24PEng8n6Yy67MqW7rnv2YeQc84-ccbrz4y1ZdHUSnxs5WXLqqoqHl6RFWetKgRX5WuyekbektOUfjPGGinlCTlRqqqlkivybwMeaDZxgIx-oKGnX3-sN_yKrjsf4mRGzAiJoqcT2K3xaM1IU47gh7ylxjtqYRzphEM0GYNfyDme7z2Co7DDvIURzRPqYR_DGAa01MEwbz503pE3vRkTvD-eZ-TX7c3P67vi_vvm2_X6vrCyZLkA3jHDbSNrU9ZCiNqwxpVdIxrZKe56AFZZzmXdy8rKhjsBXHDnKgWOOauqM_LhMHcXw-MeUtYTpuX5xkPYJ61UKZqyrWdQHEAbQ0oRer2LOJn4V3OmF_l6MasXs7qV-km-fphrF8f5-24C91w62p7zL4cc5k_-QYg6WQRvwWEEm7UL-PKC_1tnlcI</recordid><startdate>19950421</startdate><enddate>19950421</enddate><creator>Guo, Lifei</creator><creator>Degenstein, Linda</creator><creator>Dowling, James</creator><creator>Yu, Qian-Chun</creator><creator>Wollmann, Robert</creator><creator>Perman, Benjamin</creator><creator>Fuchs, Elaine</creator><general>Elsevier Inc</general><scope>6I.</scope><scope>AAFTH</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>19950421</creationdate><title>Gene targeting of BPAG1: Abnormalities in mechanical strength and cell migration in stratified epithelia and neurologic degeneration</title><author>Guo, Lifei ; Degenstein, Linda ; Dowling, James ; Yu, Qian-Chun ; Wollmann, Robert ; Perman, Benjamin ; Fuchs, Elaine</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c520t-e1b0a1c856a264446a08d2b8485b71dfee03c1156f53c581d4e141dd37ed0dc73</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1995</creationdate><topic>Animals</topic><topic>Autoantigens - genetics</topic><topic>Autoantigens - isolation & purification</topic><topic>Carrier Proteins</topic><topic>Cell Movement</topic><topic>Collagen</topic><topic>Collagen Type XVII</topic><topic>Cytoskeletal Proteins</topic><topic>Cytoskeleton - pathology</topic><topic>Cytoskeleton - ultrastructure</topic><topic>Desmosomes - chemistry</topic><topic>Desmosomes - pathology</topic><topic>Desmosomes - ultrastructure</topic><topic>Dystonia - genetics</topic><topic>Dystonin</topic><topic>Epithelium - abnormalities</topic><topic>Mice</topic><topic>Mice, Knockout</topic><topic>Nerve Degeneration - genetics</topic><topic>Nerve Tissue Proteins</topic><topic>Non-Fibrillar Collagens</topic><topic>Pemphigoid, Bullous - genetics</topic><topic>Phenotype</topic><topic>RNA, Messenger - analysis</topic><topic>Sensory Receptor Cells - pathology</topic><topic>Skin Abnormalities</topic><topic>Tensile Strength</topic><topic>Wound Healing - genetics</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Guo, Lifei</creatorcontrib><creatorcontrib>Degenstein, Linda</creatorcontrib><creatorcontrib>Dowling, James</creatorcontrib><creatorcontrib>Yu, Qian-Chun</creatorcontrib><creatorcontrib>Wollmann, Robert</creatorcontrib><creatorcontrib>Perman, Benjamin</creatorcontrib><creatorcontrib>Fuchs, Elaine</creatorcontrib><collection>ScienceDirect Open Access Titles</collection><collection>Elsevier:ScienceDirect:Open Access</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>Cell</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Guo, Lifei</au><au>Degenstein, Linda</au><au>Dowling, James</au><au>Yu, Qian-Chun</au><au>Wollmann, Robert</au><au>Perman, Benjamin</au><au>Fuchs, Elaine</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Gene targeting of BPAG1: Abnormalities in mechanical strength and cell migration in stratified epithelia and neurologic degeneration</atitle><jtitle>Cell</jtitle><addtitle>Cell</addtitle><date>1995-04-21</date><risdate>1995</risdate><volume>81</volume><issue>2</issue><spage>233</spage><epage>243</epage><pages>233-243</pages><issn>0092-8674</issn><eissn>1097-4172</eissn><abstract>BPAG1 is the major antigenic determinant of autoimmune sera of bullous pemphigoid (BP) patients. It is made by stratified squamous epithelia, where it localizes to the inner surface of specialized integrin-mediated adherens junctions (hemidesmosomes). To explore the function of BPAG1 and its relation to BP, we targeted the removal of the
BPAG1 gene in mice. Hemidesmosomes are otherwise normal, but they lack the inner plate and have no cytoskeleton attached. Though not affecting cell growth or substratum adhesion, this compromises mechanical integrity and influences migration. Unexpectedly, the mice also develop severe dystonia and sensory nerve degeneration typical of
dystonia musculorum (dt/df) mice. We show that in at least one other strain of
dt/dt mice,
BPAG1 gene is defective.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>7736575</pmid><doi>10.1016/0092-8674(95)90333-X</doi><tpages>11</tpages><oa>free_for_read</oa></addata></record> |
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| source | MEDLINE; Cell Press Free Archives; Elsevier ScienceDirect Journals; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals |
| subjects | Animals Autoantigens - genetics Autoantigens - isolation & purification Carrier Proteins Cell Movement Collagen Collagen Type XVII Cytoskeletal Proteins Cytoskeleton - pathology Cytoskeleton - ultrastructure Desmosomes - chemistry Desmosomes - pathology Desmosomes - ultrastructure Dystonia - genetics Dystonin Epithelium - abnormalities Mice Mice, Knockout Nerve Degeneration - genetics Nerve Tissue Proteins Non-Fibrillar Collagens Pemphigoid, Bullous - genetics Phenotype RNA, Messenger - analysis Sensory Receptor Cells - pathology Skin Abnormalities Tensile Strength Wound Healing - genetics |
| title | Gene targeting of BPAG1: Abnormalities in mechanical strength and cell migration in stratified epithelia and neurologic degeneration |
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