{"id":1647,"date":"2018-06-22T10:35:54","date_gmt":"2018-06-22T01:35:54","guid":{"rendered":"http:\/\/misterx95.myds.me\/?p=1647"},"modified":"2018-06-22T10:36:03","modified_gmt":"2018-06-22T01:36:03","slug":"2018-06-22","status":"publish","type":"post","link":"https:\/\/misterx95.myds.me\/wordpress\/?p=1647","title":{"rendered":"2018.06.22"},"content":{"rendered":"<h1>BP180 dysfunction triggers spontaneous skin inflammation in mice.<\/h1>\n<div class=\"auths\"><a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Zhang%20Y%5BAuthor%5D&amp;cauthor=true&amp;cauthor_uid=29866844\">Zhang Y<\/a><sup>1,<\/sup><sup>2<\/sup>,\u00a0<a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Hwang%20BJ%5BAuthor%5D&amp;cauthor=true&amp;cauthor_uid=29866844\">Hwang BJ<\/a><sup>3<\/sup>,\u00a0<a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Liu%20Z%5BAuthor%5D&amp;cauthor=true&amp;cauthor_uid=29866844\">Liu Z<\/a><sup>2,<\/sup><sup>4<\/sup>,\u00a0<a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Li%20N%5BAuthor%5D&amp;cauthor=true&amp;cauthor_uid=29866844\">Li N<\/a><sup>2<\/sup>,\u00a0<a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Lough%20K%5BAuthor%5D&amp;cauthor=true&amp;cauthor_uid=29866844\">Lough K<\/a><sup>5<\/sup>,\u00a0<a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Williams%20SE%5BAuthor%5D&amp;cauthor=true&amp;cauthor_uid=29866844\">Williams SE<\/a><sup>5<\/sup>,\u00a0<a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Chen%20J%5BAuthor%5D&amp;cauthor=true&amp;cauthor_uid=29866844\">Chen J<\/a><sup>2,<\/sup><sup>6<\/sup>,\u00a0<a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Burette%20SW%5BAuthor%5D&amp;cauthor=true&amp;cauthor_uid=29866844\">Burette SW<\/a><sup>2<\/sup>,\u00a0<a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Diaz%20LA%5BAuthor%5D&amp;cauthor=true&amp;cauthor_uid=29866844\">Diaz LA<\/a><sup>2<\/sup>,\u00a0<a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Su%20MA%5BAuthor%5D&amp;cauthor=true&amp;cauthor_uid=29866844\">Su MA<\/a><sup>3,<\/sup><sup>7<\/sup>,\u00a0<a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Xiao%20S%5BAuthor%5D&amp;cauthor=true&amp;cauthor_uid=29866844\">Xiao S<\/a><sup>8<\/sup>,\u00a0<a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Liu%20Z%5BAuthor%5D&amp;cauthor=true&amp;cauthor_uid=29866844\">Liu Z<\/a><sup>9,<\/sup><sup>3,<\/sup><sup>10<\/sup>.<\/div>\n<div class=\"afflist\">\n<div class=\"ui-helper-reset\"><\/div>\n<\/div>\n<div class=\"abstr\">\n<h3>Abstract<\/h3>\n<div class=\"\">\n<p>BP180, also known as collagen XVII, is a hemidesmosomal component and plays a key role in maintaining skin dermal\/epidermal adhesion. Dysfunction of BP180, either through genetic mutations in junctional epidermolysis bullosa (JEB) or autoantibody insult in bullous pemphigoid (BP), leads to subepidermal blistering accompanied by skin inflammation. However, whether BP180 is involved in skin inflammation remains unknown. To address this question, we generated a BP180-dysfunctional mouse strain and found that mice lacking functional BP180 (termed \u0394<i>NC16A<\/i>) developed spontaneous skin inflammatory disease, characterized by severe itch, defective skin barrier, infiltrating immune cells, elevated serum IgE levels, and increased expression of thymic stromal lymphopoietin (TSLP). Severe itch is independent of adaptive immunity and histamine, but dependent on increased expression of TSLP by keratinocytes. In addition, a high TSLP expression is detected in BP patients. Our data provide direct evidence showing that BP180 regulates skin inflammation independently of adaptive immunity, and BP180 dysfunction leads to a TSLP-mediated itch. The newly developed mouse strain could be a model for elucidation of disease mechanisms and development of novel therapeutic strategies for skin inflammation and BP180-related skin conditions.<\/p>\n<p><a href=\"http:\/\/misterx95.myds.me\/wordpress\/wp-content\/uploads\/2018\/06\/BP180-dysfunction-triggers-spontaneous-skin-inflammation-in-mice.pdf\">BP180 dysfunction triggers spontaneous skin inflammation in mice<\/a>.<\/p>\n<p><a href=\"http:\/\/misterx95.myds.me\/wordpress\/wp-content\/uploads\/2018\/06\/Supplementary-information.pdf\">Supplementary information<\/a>.<\/p>\n<\/div>\n<\/div>\n","protected":false},"excerpt":{"rendered":"<p>BP180 dysfunction triggers spontaneous skin inflammation in mice. Zhang Y1,2,\u00a0Hwang BJ3,\u00a0Liu Z2,4,\u00a0Li N2,\u00a0Lough K5,\u00a0Williams SE5,\u00a0Chen J2,6,\u00a0Burette SW2,\u00a0Diaz LA2,\u00a0Su MA3,7,\u00a0Xiao S8,\u00a0Liu Z9,3,10. Abstract BP180, also known as collagen XVII, is a hemidesmosomal component and plays a key role in maintaining skin dermal\/epidermal adhesion. Dysfunction of BP180, either through genetic mutations in junctional epidermolysis bullosa (JEB) or [&hellip;]<\/p>\n","protected":false},"author":7,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"site-sidebar-layout":"default","site-content-layout":"","ast-site-content-layout":"","site-content-style":"default","site-sidebar-style":"default","ast-global-header-display":"","ast-banner-title-visibility":"","ast-main-header-display":"","ast-hfb-above-header-display":"","ast-hfb-below-header-display":"","ast-hfb-mobile-header-display":"","site-post-title":"","ast-breadcrumbs-content":"","ast-featured-img":"","footer-sml-layout":"","theme-transparent-header-meta":"","adv-header-id-meta":"","stick-header-meta":"","header-above-stick-meta":"","header-main-stick-meta":"","header-below-stick-meta":"","astra-migrate-meta-layouts":"default","ast-page-background-enabled":"default","ast-page-background-meta":{"desktop":{"background-color":"var(--ast-global-color-4)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"tablet":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"mobile":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""}},"ast-content-background-meta":{"desktop":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"tablet":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"mobile":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""}}},"categories":[1],"tags":[],"_links":{"self":[{"href":"https:\/\/misterx95.myds.me\/wordpress\/index.php?rest_route=\/wp\/v2\/posts\/1647"}],"collection":[{"href":"https:\/\/misterx95.myds.me\/wordpress\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/misterx95.myds.me\/wordpress\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/misterx95.myds.me\/wordpress\/index.php?rest_route=\/wp\/v2\/users\/7"}],"replies":[{"embeddable":true,"href":"https:\/\/misterx95.myds.me\/wordpress\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=1647"}],"version-history":[{"count":1,"href":"https:\/\/misterx95.myds.me\/wordpress\/index.php?rest_route=\/wp\/v2\/posts\/1647\/revisions"}],"predecessor-version":[{"id":1650,"href":"https:\/\/misterx95.myds.me\/wordpress\/index.php?rest_route=\/wp\/v2\/posts\/1647\/revisions\/1650"}],"wp:attachment":[{"href":"https:\/\/misterx95.myds.me\/wordpress\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=1647"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/misterx95.myds.me\/wordpress\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=1647"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/misterx95.myds.me\/wordpress\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=1647"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}