Altered epidermal lipid processing and calcium distribution in the KID syndrome mouse model Cx26S17F
•Disturbed epidermal lipid secretion and location in Cx26S17F KID syndrome mouse model.•Loss of ω-esterified ceramides from the epidermal surface of Cx26S17F mice.•Cx26S17F leads to an altered epidermal calcium gradient. The keratitis–ichthyosis–deafness (KID) syndrome is caused by mutations in the...
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| Veröffentlicht in: | FEBS letters 2015-07, Vol.589 (15), p.1904-1910 |
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| creator | Bosen, Felicitas Celli, Anna Crumrine, Debra vom Dorp, Katharina Ebel, Philipp Jastrow, Holger Dörmann, Peter Winterhager, Elke Mauro, Theodora Willecke, Klaus |
| description | •Disturbed epidermal lipid secretion and location in Cx26S17F KID syndrome mouse model.•Loss of ω-esterified ceramides from the epidermal surface of Cx26S17F mice.•Cx26S17F leads to an altered epidermal calcium gradient.
The keratitis–ichthyosis–deafness (KID) syndrome is caused by mutations in the gap junctional channel protein connexin 26 (Cx26), among them the mutation Cx26S17F. Heterozygous Cx26S17F mice resemble the human KID syndrome, i.e. exhibiting epidermal hyperplasia and hearing impairments. Newborn Cx26S17F mice show a defective epidermal water barrier as well as altered epidermal lipid secretion and location. Linoleoyl ω-esterified ceramides are strongly decreased on the skin surface of Cx26S17F mice. Moreover, the epidermal calcium gradient is altered in the mutant mice. These alterations may be caused by an abnormal Cx26S17F channel function that leads to a defective epidermal water barrier, which in turn may trigger the hyperproliferation seen in the KID syndrome. |
| doi_str_mv | 10.1016/j.febslet.2015.05.047 |
| format | Article |
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The keratitis–ichthyosis–deafness (KID) syndrome is caused by mutations in the gap junctional channel protein connexin 26 (Cx26), among them the mutation Cx26S17F. Heterozygous Cx26S17F mice resemble the human KID syndrome, i.e. exhibiting epidermal hyperplasia and hearing impairments. Newborn Cx26S17F mice show a defective epidermal water barrier as well as altered epidermal lipid secretion and location. Linoleoyl ω-esterified ceramides are strongly decreased on the skin surface of Cx26S17F mice. Moreover, the epidermal calcium gradient is altered in the mutant mice. These alterations may be caused by an abnormal Cx26S17F channel function that leads to a defective epidermal water barrier, which in turn may trigger the hyperproliferation seen in the KID syndrome.</description><identifier>ISSN: 0014-5793</identifier><identifier>EISSN: 1873-3468</identifier><identifier>DOI: 10.1016/j.febslet.2015.05.047</identifier><identifier>PMID: 26070424</identifier><language>eng</language><publisher>England: Elsevier B.V</publisher><subject>animal models ; Animals ; calcium ; Calcium - metabolism ; ceramides ; Connexin 26 ; Connexins - genetics ; Deafness - metabolism ; Disease Models, Animal ; Epidermal calcium gradient ; Epidermal ceramides ; Epidermal water barrier defect ; Epidermis - metabolism ; Female ; hearing ; heterozygosity ; humans ; hyperplasia ; Ichthyosis - metabolism ; Keratitis - metabolism ; Keratitis–ichthyosis–deafness syndrome ; Lipid Metabolism ; Male ; Mice ; Microscopy, Fluorescence ; mutants ; mutation ; neonates ; secretion ; Transgenic mouse mutant</subject><ispartof>FEBS letters, 2015-07, Vol.589 (15), p.1904-1910</ispartof><rights>2015 Federation of European Biochemical Societies</rights><rights>FEBS Letters 589 (2015) 1873-3468 © 2015 Federation of European Biochemical Societies</rights><rights>Copyright © 2015 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c5982-5b6f7b58192a433296ac9026c9b81e72e74db970eb41a5c1e05ab6b2af8696493</citedby><cites>FETCH-LOGICAL-c5982-5b6f7b58192a433296ac9026c9b81e72e74db970eb41a5c1e05ab6b2af8696493</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1016%2Fj.febslet.2015.05.047$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S0014579315004482$$EHTML$$P50$$Gelsevier$$Hfree_for_read</linktohtml><link.rule.ids>230,314,776,780,881,1411,1427,3537,27901,27902,45550,45551,46384,46808,65306</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/26070424$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Bosen, Felicitas</creatorcontrib><creatorcontrib>Celli, Anna</creatorcontrib><creatorcontrib>Crumrine, Debra</creatorcontrib><creatorcontrib>vom Dorp, Katharina</creatorcontrib><creatorcontrib>Ebel, Philipp</creatorcontrib><creatorcontrib>Jastrow, Holger</creatorcontrib><creatorcontrib>Dörmann, Peter</creatorcontrib><creatorcontrib>Winterhager, Elke</creatorcontrib><creatorcontrib>Mauro, Theodora</creatorcontrib><creatorcontrib>Willecke, Klaus</creatorcontrib><title>Altered epidermal lipid processing and calcium distribution in the KID syndrome mouse model Cx26S17F</title><title>FEBS letters</title><addtitle>FEBS Lett</addtitle><description>•Disturbed epidermal lipid secretion and location in Cx26S17F KID syndrome mouse model.•Loss of ω-esterified ceramides from the epidermal surface of Cx26S17F mice.•Cx26S17F leads to an altered epidermal calcium gradient.
The keratitis–ichthyosis–deafness (KID) syndrome is caused by mutations in the gap junctional channel protein connexin 26 (Cx26), among them the mutation Cx26S17F. Heterozygous Cx26S17F mice resemble the human KID syndrome, i.e. exhibiting epidermal hyperplasia and hearing impairments. Newborn Cx26S17F mice show a defective epidermal water barrier as well as altered epidermal lipid secretion and location. Linoleoyl ω-esterified ceramides are strongly decreased on the skin surface of Cx26S17F mice. Moreover, the epidermal calcium gradient is altered in the mutant mice. These alterations may be caused by an abnormal Cx26S17F channel function that leads to a defective epidermal water barrier, which in turn may trigger the hyperproliferation seen in the KID syndrome.</description><subject>animal models</subject><subject>Animals</subject><subject>calcium</subject><subject>Calcium - metabolism</subject><subject>ceramides</subject><subject>Connexin 26</subject><subject>Connexins - genetics</subject><subject>Deafness - metabolism</subject><subject>Disease Models, Animal</subject><subject>Epidermal calcium gradient</subject><subject>Epidermal ceramides</subject><subject>Epidermal water barrier defect</subject><subject>Epidermis - metabolism</subject><subject>Female</subject><subject>hearing</subject><subject>heterozygosity</subject><subject>humans</subject><subject>hyperplasia</subject><subject>Ichthyosis - metabolism</subject><subject>Keratitis - metabolism</subject><subject>Keratitis–ichthyosis–deafness syndrome</subject><subject>Lipid Metabolism</subject><subject>Male</subject><subject>Mice</subject><subject>Microscopy, Fluorescence</subject><subject>mutants</subject><subject>mutation</subject><subject>neonates</subject><subject>secretion</subject><subject>Transgenic mouse mutant</subject><issn>0014-5793</issn><issn>1873-3468</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2015</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNUsFu1DAQtRCILoVPAPnIJVvbcez4AmqXLq2oxKFwthx70nrlJIudFPbvcdhtBadFGtlj-c3Tm3mD0FtKlpRQcbZZttCkAOOSEVotSQ4un6EFrWVZlFzUz9GCEMqLSqryBL1KaUPyu6bqJTphgkjCGV8gdx5GiOAwbL2D2JmAg88p3sbBQkq-v8Omd9iaYP3UYefTGH0zjX7ose_xeA_4y_UnnHa9i0MHuBumNJ8OAl79YuKWyvVr9KI1IcGbw32Kvq8vv62uipuvn69X5zeFrVTNiqoRrWyqLJEZXpZMCWMVYcKqpqYgGUjuGiUJNJyaylIglWlEw0xbCyW4Kk_Rhz3vdmo6cBb6MZqgt9F3Ju70YLz-96f39_pueNBccspqlgneHwji8GOCNOrOJwshmB5yX5rlETJW5vEehVKZPagJr-rjUKG4JIJzkqHVHmrjkFKE9kk8JXr2XW_0wXc9-65JDi5z3bu_O3-qejQ6A672gJ8-wO7_WPX68oLdzks07xCtCOH8z5A-7qkgW_ngIepkPfQWnI9gR-0Gf0Ttb9US1mo</recordid><startdate>20150708</startdate><enddate>20150708</enddate><creator>Bosen, Felicitas</creator><creator>Celli, Anna</creator><creator>Crumrine, Debra</creator><creator>vom Dorp, Katharina</creator><creator>Ebel, Philipp</creator><creator>Jastrow, Holger</creator><creator>Dörmann, Peter</creator><creator>Winterhager, Elke</creator><creator>Mauro, Theodora</creator><creator>Willecke, Klaus</creator><general>Elsevier B.V</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><scope>7QP</scope><scope>7S9</scope><scope>L.6</scope><scope>5PM</scope></search><sort><creationdate>20150708</creationdate><title>Altered epidermal lipid processing and calcium distribution in the KID syndrome mouse model Cx26S17F</title><author>Bosen, Felicitas ; Celli, Anna ; Crumrine, Debra ; vom Dorp, Katharina ; Ebel, Philipp ; Jastrow, Holger ; Dörmann, Peter ; Winterhager, Elke ; Mauro, Theodora ; Willecke, Klaus</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c5982-5b6f7b58192a433296ac9026c9b81e72e74db970eb41a5c1e05ab6b2af8696493</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2015</creationdate><topic>animal models</topic><topic>Animals</topic><topic>calcium</topic><topic>Calcium - metabolism</topic><topic>ceramides</topic><topic>Connexin 26</topic><topic>Connexins - genetics</topic><topic>Deafness - metabolism</topic><topic>Disease Models, Animal</topic><topic>Epidermal calcium gradient</topic><topic>Epidermal ceramides</topic><topic>Epidermal water barrier defect</topic><topic>Epidermis - metabolism</topic><topic>Female</topic><topic>hearing</topic><topic>heterozygosity</topic><topic>humans</topic><topic>hyperplasia</topic><topic>Ichthyosis - metabolism</topic><topic>Keratitis - metabolism</topic><topic>Keratitis–ichthyosis–deafness syndrome</topic><topic>Lipid Metabolism</topic><topic>Male</topic><topic>Mice</topic><topic>Microscopy, Fluorescence</topic><topic>mutants</topic><topic>mutation</topic><topic>neonates</topic><topic>secretion</topic><topic>Transgenic mouse mutant</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Bosen, Felicitas</creatorcontrib><creatorcontrib>Celli, Anna</creatorcontrib><creatorcontrib>Crumrine, Debra</creatorcontrib><creatorcontrib>vom Dorp, Katharina</creatorcontrib><creatorcontrib>Ebel, Philipp</creatorcontrib><creatorcontrib>Jastrow, Holger</creatorcontrib><creatorcontrib>Dörmann, Peter</creatorcontrib><creatorcontrib>Winterhager, Elke</creatorcontrib><creatorcontrib>Mauro, Theodora</creatorcontrib><creatorcontrib>Willecke, Klaus</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><collection>Calcium & Calcified Tissue Abstracts</collection><collection>AGRICOLA</collection><collection>AGRICOLA - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>FEBS letters</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Bosen, Felicitas</au><au>Celli, Anna</au><au>Crumrine, Debra</au><au>vom Dorp, Katharina</au><au>Ebel, Philipp</au><au>Jastrow, Holger</au><au>Dörmann, Peter</au><au>Winterhager, Elke</au><au>Mauro, Theodora</au><au>Willecke, Klaus</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Altered epidermal lipid processing and calcium distribution in the KID syndrome mouse model Cx26S17F</atitle><jtitle>FEBS letters</jtitle><addtitle>FEBS Lett</addtitle><date>2015-07-08</date><risdate>2015</risdate><volume>589</volume><issue>15</issue><spage>1904</spage><epage>1910</epage><pages>1904-1910</pages><issn>0014-5793</issn><eissn>1873-3468</eissn><abstract>•Disturbed epidermal lipid secretion and location in Cx26S17F KID syndrome mouse model.•Loss of ω-esterified ceramides from the epidermal surface of Cx26S17F mice.•Cx26S17F leads to an altered epidermal calcium gradient.
The keratitis–ichthyosis–deafness (KID) syndrome is caused by mutations in the gap junctional channel protein connexin 26 (Cx26), among them the mutation Cx26S17F. Heterozygous Cx26S17F mice resemble the human KID syndrome, i.e. exhibiting epidermal hyperplasia and hearing impairments. Newborn Cx26S17F mice show a defective epidermal water barrier as well as altered epidermal lipid secretion and location. Linoleoyl ω-esterified ceramides are strongly decreased on the skin surface of Cx26S17F mice. Moreover, the epidermal calcium gradient is altered in the mutant mice. These alterations may be caused by an abnormal Cx26S17F channel function that leads to a defective epidermal water barrier, which in turn may trigger the hyperproliferation seen in the KID syndrome.</abstract><cop>England</cop><pub>Elsevier B.V</pub><pmid>26070424</pmid><doi>10.1016/j.febslet.2015.05.047</doi><tpages>7</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | animal models Animals calcium Calcium - metabolism ceramides Connexin 26 Connexins - genetics Deafness - metabolism Disease Models, Animal Epidermal calcium gradient Epidermal ceramides Epidermal water barrier defect Epidermis - metabolism Female hearing heterozygosity humans hyperplasia Ichthyosis - metabolism Keratitis - metabolism Keratitis–ichthyosis–deafness syndrome Lipid Metabolism Male Mice Microscopy, Fluorescence mutants mutation neonates secretion Transgenic mouse mutant |
| title | Altered epidermal lipid processing and calcium distribution in the KID syndrome mouse model Cx26S17F |
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