Fibroblast activation and ECM remodelling in genodermatoses
Summary Recessive dystrophic epidermolysis bullosa (RDEB), Kindler syndrome (KS) and Xeroderma pigmentosum C (XPC) are three genetic skin diseases (i.e. genodermatoses) in which patients are at increased risk of developing cancer. The events leading to cancer in these diseases, including those takin...
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| Veröffentlicht in: | British journal of dermatology (1951) 2019-09, Vol.181 (3), p.e66-e66 |
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| creator | Chacón‐Solano, E. León, C. Díaz, F. García‐García, F. García, M. Escámez, M.J. Guerrero‐Aspizua, S. Conti, C.J. Mencía, Á. Martínez‐Santamaría, L. Llames, S. Pévida, M. Carbonell‐Caballero, J. Puig‐Butillé, J.A. Maseda, R. Puig, S. Lucas, R. Baselga, E. Larcher, F. Dopazo, J. Río, M. |
| description | Summary
Recessive dystrophic epidermolysis bullosa (RDEB), Kindler syndrome (KS) and Xeroderma pigmentosum C (XPC) are three genetic skin diseases (i.e. genodermatoses) in which patients are at increased risk of developing cancer. The events leading to cancer in these diseases, including those taking place at the level of the skin, are still not well understood even though they are under intense investigation. To deepen the knowledge of these rare diseases with the ultimate goal of finding new treatments, the Spanish research group investigated the gene expression profile (signature) of a type of skin cell called dermal fibroblasts from patients with RDEB, KS and XPC. The researchers found that the fibroblasts from three genodermatoses share a large number of genes aberrantly (wrongly) expressed in a similar fashion. That in turn translates into what is defined as an activated phenotype and leads to changes in dermal (a skin layer) stiffness and an imbalance of the redox (a type of chemical reaction) status. This overlapping signature seems not to depend on the primary disease‐causing deficiency defect (i.e. loss of type VII collagen, kindlin‐1 or XPC) but rather the consequence of a common injury‐responsive event not yet fully understood. In conclusion, this study adds to the current knowledge about the role played by dermal fibroblasts in RDEB, XPC and KS and highlights new targets for possible treatments.
Linked Article: Chacón‐Solano et al. Br J Dermatol 2019; 181:512–522 |
| doi_str_mv | 10.1111/bjd.18271 |
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Recessive dystrophic epidermolysis bullosa (RDEB), Kindler syndrome (KS) and Xeroderma pigmentosum C (XPC) are three genetic skin diseases (i.e. genodermatoses) in which patients are at increased risk of developing cancer. The events leading to cancer in these diseases, including those taking place at the level of the skin, are still not well understood even though they are under intense investigation. To deepen the knowledge of these rare diseases with the ultimate goal of finding new treatments, the Spanish research group investigated the gene expression profile (signature) of a type of skin cell called dermal fibroblasts from patients with RDEB, KS and XPC. The researchers found that the fibroblasts from three genodermatoses share a large number of genes aberrantly (wrongly) expressed in a similar fashion. That in turn translates into what is defined as an activated phenotype and leads to changes in dermal (a skin layer) stiffness and an imbalance of the redox (a type of chemical reaction) status. This overlapping signature seems not to depend on the primary disease‐causing deficiency defect (i.e. loss of type VII collagen, kindlin‐1 or XPC) but rather the consequence of a common injury‐responsive event not yet fully understood. In conclusion, this study adds to the current knowledge about the role played by dermal fibroblasts in RDEB, XPC and KS and highlights new targets for possible treatments.
Linked Article: Chacón‐Solano et al. Br J Dermatol 2019; 181:512–522</description><identifier>ISSN: 0007-0963</identifier><identifier>EISSN: 1365-2133</identifier><identifier>DOI: 10.1111/bjd.18271</identifier><language>eng</language><publisher>Oxford: Oxford University Press</publisher><subject>Collagen ; Dystrophic epidermolysis bullosa ; Epidermolysis bullosa ; Extracellular matrix ; Fibroblasts ; Gene expression ; Phenotypes ; Skin diseases ; Xeroderma pigmentosum ; XPC protein</subject><ispartof>British journal of dermatology (1951), 2019-09, Vol.181 (3), p.e66-e66</ispartof><rights>2019 British Association of Dermatologists</rights><rights>Copyright © 2019 British Association of Dermatologists</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fbjd.18271$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fbjd.18271$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids></links><search><creatorcontrib>Chacón‐Solano, E.</creatorcontrib><creatorcontrib>León, C.</creatorcontrib><creatorcontrib>Díaz, F.</creatorcontrib><creatorcontrib>García‐García, F.</creatorcontrib><creatorcontrib>García, M.</creatorcontrib><creatorcontrib>Escámez, M.J.</creatorcontrib><creatorcontrib>Guerrero‐Aspizua, S.</creatorcontrib><creatorcontrib>Conti, C.J.</creatorcontrib><creatorcontrib>Mencía, Á.</creatorcontrib><creatorcontrib>Martínez‐Santamaría, L.</creatorcontrib><creatorcontrib>Llames, S.</creatorcontrib><creatorcontrib>Pévida, M.</creatorcontrib><creatorcontrib>Carbonell‐Caballero, J.</creatorcontrib><creatorcontrib>Puig‐Butillé, J.A.</creatorcontrib><creatorcontrib>Maseda, R.</creatorcontrib><creatorcontrib>Puig, S.</creatorcontrib><creatorcontrib>Lucas, R.</creatorcontrib><creatorcontrib>Baselga, E.</creatorcontrib><creatorcontrib>Larcher, F.</creatorcontrib><creatorcontrib>Dopazo, J.</creatorcontrib><creatorcontrib>Río, M.</creatorcontrib><title>Fibroblast activation and ECM remodelling in genodermatoses</title><title>British journal of dermatology (1951)</title><description>Summary
Recessive dystrophic epidermolysis bullosa (RDEB), Kindler syndrome (KS) and Xeroderma pigmentosum C (XPC) are three genetic skin diseases (i.e. genodermatoses) in which patients are at increased risk of developing cancer. The events leading to cancer in these diseases, including those taking place at the level of the skin, are still not well understood even though they are under intense investigation. To deepen the knowledge of these rare diseases with the ultimate goal of finding new treatments, the Spanish research group investigated the gene expression profile (signature) of a type of skin cell called dermal fibroblasts from patients with RDEB, KS and XPC. The researchers found that the fibroblasts from three genodermatoses share a large number of genes aberrantly (wrongly) expressed in a similar fashion. That in turn translates into what is defined as an activated phenotype and leads to changes in dermal (a skin layer) stiffness and an imbalance of the redox (a type of chemical reaction) status. This overlapping signature seems not to depend on the primary disease‐causing deficiency defect (i.e. loss of type VII collagen, kindlin‐1 or XPC) but rather the consequence of a common injury‐responsive event not yet fully understood. In conclusion, this study adds to the current knowledge about the role played by dermal fibroblasts in RDEB, XPC and KS and highlights new targets for possible treatments.
Linked Article: Chacón‐Solano et al. Br J Dermatol 2019; 181:512–522</description><subject>Collagen</subject><subject>Dystrophic epidermolysis bullosa</subject><subject>Epidermolysis bullosa</subject><subject>Extracellular matrix</subject><subject>Fibroblasts</subject><subject>Gene expression</subject><subject>Phenotypes</subject><subject>Skin diseases</subject><subject>Xeroderma pigmentosum</subject><subject>XPC protein</subject><issn>0007-0963</issn><issn>1365-2133</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><recordid>eNp1kE1PwzAMQCMEEmVw4B9U4sShWxJnTSpOUDY-NMQFzlGapFOmrhlJB9q_J1Cu-GLZerblh9AlwVOSYtZszJQIyskRygiU84ISgGOUYYx5gasSTtFZjBuMCeA5ztDN0jXBN52KQ6704D7V4Hyfq97ki_olD3brje06169z1-dr26cybNXgo43n6KRVXbQXf3mC3peLt_qxWL0-PNW3q0IT4KQgjAuwTVsBU6xKPVEC41pzpiw2woLRhkGlqaHElLTimmAtNBbKKADRwARdjXt3wX_sbRzkxu9Dn05KSkX6AyrBEnU9Ujr4GINt5S64rQoHSbD8cSOTG_nrJrGzkf1ynT38D8q75_tx4hvCT2Qy</recordid><startdate>201909</startdate><enddate>201909</enddate><creator>Chacón‐Solano, E.</creator><creator>León, C.</creator><creator>Díaz, F.</creator><creator>García‐García, F.</creator><creator>García, M.</creator><creator>Escámez, M.J.</creator><creator>Guerrero‐Aspizua, S.</creator><creator>Conti, C.J.</creator><creator>Mencía, Á.</creator><creator>Martínez‐Santamaría, L.</creator><creator>Llames, S.</creator><creator>Pévida, M.</creator><creator>Carbonell‐Caballero, J.</creator><creator>Puig‐Butillé, J.A.</creator><creator>Maseda, R.</creator><creator>Puig, S.</creator><creator>Lucas, R.</creator><creator>Baselga, E.</creator><creator>Larcher, F.</creator><creator>Dopazo, J.</creator><creator>Río, M.</creator><general>Oxford University Press</general><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>K9.</scope><scope>NAPCQ</scope></search><sort><creationdate>201909</creationdate><title>Fibroblast activation and ECM remodelling in genodermatoses</title><author>Chacón‐Solano, E. ; León, C. ; Díaz, F. ; García‐García, F. ; García, M. ; Escámez, M.J. ; Guerrero‐Aspizua, S. ; Conti, C.J. ; Mencía, Á. ; Martínez‐Santamaría, L. ; Llames, S. ; Pévida, M. ; Carbonell‐Caballero, J. ; Puig‐Butillé, J.A. ; Maseda, R. ; Puig, S. ; Lucas, R. ; Baselga, E. ; Larcher, F. ; Dopazo, J. ; Río, M.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c1371-14783ebf934a49c1386347cc74ae0d8e3dcd439c2d21d6297c10c8c08ada338b3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2019</creationdate><topic>Collagen</topic><topic>Dystrophic epidermolysis bullosa</topic><topic>Epidermolysis bullosa</topic><topic>Extracellular matrix</topic><topic>Fibroblasts</topic><topic>Gene expression</topic><topic>Phenotypes</topic><topic>Skin diseases</topic><topic>Xeroderma pigmentosum</topic><topic>XPC protein</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Chacón‐Solano, E.</creatorcontrib><creatorcontrib>León, C.</creatorcontrib><creatorcontrib>Díaz, F.</creatorcontrib><creatorcontrib>García‐García, F.</creatorcontrib><creatorcontrib>García, M.</creatorcontrib><creatorcontrib>Escámez, M.J.</creatorcontrib><creatorcontrib>Guerrero‐Aspizua, S.</creatorcontrib><creatorcontrib>Conti, C.J.</creatorcontrib><creatorcontrib>Mencía, Á.</creatorcontrib><creatorcontrib>Martínez‐Santamaría, L.</creatorcontrib><creatorcontrib>Llames, S.</creatorcontrib><creatorcontrib>Pévida, M.</creatorcontrib><creatorcontrib>Carbonell‐Caballero, J.</creatorcontrib><creatorcontrib>Puig‐Butillé, J.A.</creatorcontrib><creatorcontrib>Maseda, R.</creatorcontrib><creatorcontrib>Puig, S.</creatorcontrib><creatorcontrib>Lucas, R.</creatorcontrib><creatorcontrib>Baselga, E.</creatorcontrib><creatorcontrib>Larcher, F.</creatorcontrib><creatorcontrib>Dopazo, J.</creatorcontrib><creatorcontrib>Río, M.</creatorcontrib><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Premium</collection><jtitle>British journal of dermatology (1951)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Chacón‐Solano, E.</au><au>León, C.</au><au>Díaz, F.</au><au>García‐García, F.</au><au>García, M.</au><au>Escámez, M.J.</au><au>Guerrero‐Aspizua, S.</au><au>Conti, C.J.</au><au>Mencía, Á.</au><au>Martínez‐Santamaría, L.</au><au>Llames, S.</au><au>Pévida, M.</au><au>Carbonell‐Caballero, J.</au><au>Puig‐Butillé, J.A.</au><au>Maseda, R.</au><au>Puig, S.</au><au>Lucas, R.</au><au>Baselga, E.</au><au>Larcher, F.</au><au>Dopazo, J.</au><au>Río, M.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Fibroblast activation and ECM remodelling in genodermatoses</atitle><jtitle>British journal of dermatology (1951)</jtitle><date>2019-09</date><risdate>2019</risdate><volume>181</volume><issue>3</issue><spage>e66</spage><epage>e66</epage><pages>e66-e66</pages><issn>0007-0963</issn><eissn>1365-2133</eissn><abstract>Summary
Recessive dystrophic epidermolysis bullosa (RDEB), Kindler syndrome (KS) and Xeroderma pigmentosum C (XPC) are three genetic skin diseases (i.e. genodermatoses) in which patients are at increased risk of developing cancer. The events leading to cancer in these diseases, including those taking place at the level of the skin, are still not well understood even though they are under intense investigation. To deepen the knowledge of these rare diseases with the ultimate goal of finding new treatments, the Spanish research group investigated the gene expression profile (signature) of a type of skin cell called dermal fibroblasts from patients with RDEB, KS and XPC. The researchers found that the fibroblasts from three genodermatoses share a large number of genes aberrantly (wrongly) expressed in a similar fashion. That in turn translates into what is defined as an activated phenotype and leads to changes in dermal (a skin layer) stiffness and an imbalance of the redox (a type of chemical reaction) status. This overlapping signature seems not to depend on the primary disease‐causing deficiency defect (i.e. loss of type VII collagen, kindlin‐1 or XPC) but rather the consequence of a common injury‐responsive event not yet fully understood. In conclusion, this study adds to the current knowledge about the role played by dermal fibroblasts in RDEB, XPC and KS and highlights new targets for possible treatments.
Linked Article: Chacón‐Solano et al. Br J Dermatol 2019; 181:512–522</abstract><cop>Oxford</cop><pub>Oxford University Press</pub><doi>10.1111/bjd.18271</doi><tpages>1</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Collagen Dystrophic epidermolysis bullosa Epidermolysis bullosa Extracellular matrix Fibroblasts Gene expression Phenotypes Skin diseases Xeroderma pigmentosum XPC protein |
| title | Fibroblast activation and ECM remodelling in genodermatoses |
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