Eintrag weiter verarbeiten
Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa
Gespeichert in:
| Zeitschriftentitel: | International Journal of Molecular Sciences |
|---|---|
| Personen und Körperschaften: | , , , |
| In: | International Journal of Molecular Sciences, 22, 2021, 10, S. 5104 |
| Format: | E-Article |
| Sprache: | Englisch |
| veröffentlicht: |
MDPI AG
|
| Schlagwörter: |
| author_facet |
Tartaglia, Grace Cao, Qingqing Padron, Zachary M. South, Andrew P. Tartaglia, Grace Cao, Qingqing Padron, Zachary M. South, Andrew P. |
|---|---|
| author |
Tartaglia, Grace Cao, Qingqing Padron, Zachary M. South, Andrew P. |
| spellingShingle |
Tartaglia, Grace Cao, Qingqing Padron, Zachary M. South, Andrew P. International Journal of Molecular Sciences Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa Inorganic Chemistry Organic Chemistry Physical and Theoretical Chemistry Computer Science Applications Spectroscopy Molecular Biology General Medicine Catalysis |
| author_sort |
tartaglia, grace |
| spelling |
Tartaglia, Grace Cao, Qingqing Padron, Zachary M. South, Andrew P. 1422-0067 MDPI AG Inorganic Chemistry Organic Chemistry Physical and Theoretical Chemistry Computer Science Applications Spectroscopy Molecular Biology General Medicine Catalysis http://dx.doi.org/10.3390/ijms22105104 <jats:p>Recessive Dystrophic Epidermolysis Bullosa (RDEB) is a devastating skin blistering disease caused by mutations in the gene encoding type VII collagen (C7), leading to epidermal fragility, trauma-induced blistering, and long term, hard-to-heal wounds. Fibrosis develops rapidly in RDEB skin and contributes to both chronic wounds, which emerge after cycles of repetitive wound and scar formation, and squamous cell carcinoma—the single biggest cause of death in this patient group. The molecular pathways disrupted in a broad spectrum of fibrotic disease are also disrupted in RDEB, and squamous cell carcinomas arising in RDEB are thus far molecularly indistinct from other sub-types of aggressive squamous cell carcinoma (SCC). Collectively these data demonstrate RDEB is a model for understanding the molecular basis of both fibrosis and rapidly developing aggressive cancer. A number of studies have shown that RDEB pathogenesis is driven by a radical change in extracellular matrix (ECM) composition and increased transforming growth factor-beta (TGFβ) signaling that is a direct result of C7 loss-of-function in dermal fibroblasts. However, the exact mechanism of how C7 loss results in extensive fibrosis is unclear, particularly how TGFβ signaling is activated and then sustained through complex networks of cell-cell interaction not limited to the traditional fibrotic protagonist, the dermal fibroblast. Continued study of this rare disease will likely yield paradigms relevant to more common pathologies.</jats:p> Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa International Journal of Molecular Sciences |
| doi_str_mv |
10.3390/ijms22105104 |
| facet_avail |
Online Free |
| finc_class_facet |
Chemie und Pharmazie Physik Informatik Biologie |
| format |
ElectronicArticle |
| fullrecord |
blob:ai-49-aHR0cDovL2R4LmRvaS5vcmcvMTAuMzM5MC9pam1zMjIxMDUxMDQ |
| id |
ai-49-aHR0cDovL2R4LmRvaS5vcmcvMTAuMzM5MC9pam1zMjIxMDUxMDQ |
| institution |
DE-Gla1 DE-Zi4 DE-15 DE-Rs1 DE-Pl11 DE-105 DE-14 DE-Ch1 DE-L229 DE-D275 DE-Bn3 DE-Brt1 DE-Zwi2 DE-D161 |
| imprint |
MDPI AG, 2021 |
| imprint_str_mv |
MDPI AG, 2021 |
| issn |
1422-0067 |
| issn_str_mv |
1422-0067 |
| language |
English |
| mega_collection |
MDPI AG (CrossRef) |
| match_str |
tartaglia2021impairedwoundhealingfibrosisandcancertheparadigmofrecessivedystrophicepidermolysisbullosa |
| publishDateSort |
2021 |
| publisher |
MDPI AG |
| recordtype |
ai |
| record_format |
ai |
| series |
International Journal of Molecular Sciences |
| source_id |
49 |
| title |
Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa |
| title_unstemmed |
Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa |
| title_full |
Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa |
| title_fullStr |
Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa |
| title_full_unstemmed |
Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa |
| title_short |
Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa |
| title_sort |
impaired wound healing, fibrosis, and cancer: the paradigm of recessive dystrophic epidermolysis bullosa |
| topic |
Inorganic Chemistry Organic Chemistry Physical and Theoretical Chemistry Computer Science Applications Spectroscopy Molecular Biology General Medicine Catalysis |
| url |
http://dx.doi.org/10.3390/ijms22105104 |
| publishDate |
2021 |
| physical |
5104 |
| description |
<jats:p>Recessive Dystrophic Epidermolysis Bullosa (RDEB) is a devastating skin blistering disease caused by mutations in the gene encoding type VII collagen (C7), leading to epidermal fragility, trauma-induced blistering, and long term, hard-to-heal wounds. Fibrosis develops rapidly in RDEB skin and contributes to both chronic wounds, which emerge after cycles of repetitive wound and scar formation, and squamous cell carcinoma—the single biggest cause of death in this patient group. The molecular pathways disrupted in a broad spectrum of fibrotic disease are also disrupted in RDEB, and squamous cell carcinomas arising in RDEB are thus far molecularly indistinct from other sub-types of aggressive squamous cell carcinoma (SCC). Collectively these data demonstrate RDEB is a model for understanding the molecular basis of both fibrosis and rapidly developing aggressive cancer. A number of studies have shown that RDEB pathogenesis is driven by a radical change in extracellular matrix (ECM) composition and increased transforming growth factor-beta (TGFβ) signaling that is a direct result of C7 loss-of-function in dermal fibroblasts. However, the exact mechanism of how C7 loss results in extensive fibrosis is unclear, particularly how TGFβ signaling is activated and then sustained through complex networks of cell-cell interaction not limited to the traditional fibrotic protagonist, the dermal fibroblast. Continued study of this rare disease will likely yield paradigms relevant to more common pathologies.</jats:p> |
| container_issue |
10 |
| container_start_page |
0 |
| container_title |
International Journal of Molecular Sciences |
| container_volume |
22 |
| format_de105 |
Article, E-Article |
| format_de14 |
Article, E-Article |
| format_de15 |
Article, E-Article |
| format_de520 |
Article, E-Article |
| format_de540 |
Article, E-Article |
| format_dech1 |
Article, E-Article |
| format_ded117 |
Article, E-Article |
| format_degla1 |
E-Article |
| format_del152 |
Buch |
| format_del189 |
Article, E-Article |
| format_dezi4 |
Article |
| format_dezwi2 |
Article, E-Article |
| format_finc |
Article, E-Article |
| format_nrw |
Article, E-Article |
| _version_ |
1792347358146592771 |
| geogr_code |
not assigned |
| last_indexed |
2024-03-01T17:53:30.46Z |
| geogr_code_person |
not assigned |
| openURL |
url_ver=Z39.88-2004&ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fvufind.svn.sourceforge.net%3Agenerator&rft.title=Impaired+Wound+Healing%2C+Fibrosis%2C+and+Cancer%3A+The+Paradigm+of+Recessive+Dystrophic+Epidermolysis+Bullosa&rft.date=2021-05-12&genre=article&issn=1422-0067&volume=22&issue=10&pages=5104&jtitle=International+Journal+of+Molecular+Sciences&atitle=Impaired+Wound+Healing%2C+Fibrosis%2C+and+Cancer%3A+The+Paradigm+of+Recessive+Dystrophic+Epidermolysis+Bullosa&aulast=South&aufirst=Andrew+P.&rft_id=info%3Adoi%2F10.3390%2Fijms22105104&rft.language%5B0%5D=eng |
| SOLR | |
| _version_ | 1792347358146592771 |
| author | Tartaglia, Grace, Cao, Qingqing, Padron, Zachary M., South, Andrew P. |
| author_facet | Tartaglia, Grace, Cao, Qingqing, Padron, Zachary M., South, Andrew P., Tartaglia, Grace, Cao, Qingqing, Padron, Zachary M., South, Andrew P. |
| author_sort | tartaglia, grace |
| container_issue | 10 |
| container_start_page | 0 |
| container_title | International Journal of Molecular Sciences |
| container_volume | 22 |
| description | <jats:p>Recessive Dystrophic Epidermolysis Bullosa (RDEB) is a devastating skin blistering disease caused by mutations in the gene encoding type VII collagen (C7), leading to epidermal fragility, trauma-induced blistering, and long term, hard-to-heal wounds. Fibrosis develops rapidly in RDEB skin and contributes to both chronic wounds, which emerge after cycles of repetitive wound and scar formation, and squamous cell carcinoma—the single biggest cause of death in this patient group. The molecular pathways disrupted in a broad spectrum of fibrotic disease are also disrupted in RDEB, and squamous cell carcinomas arising in RDEB are thus far molecularly indistinct from other sub-types of aggressive squamous cell carcinoma (SCC). Collectively these data demonstrate RDEB is a model for understanding the molecular basis of both fibrosis and rapidly developing aggressive cancer. A number of studies have shown that RDEB pathogenesis is driven by a radical change in extracellular matrix (ECM) composition and increased transforming growth factor-beta (TGFβ) signaling that is a direct result of C7 loss-of-function in dermal fibroblasts. However, the exact mechanism of how C7 loss results in extensive fibrosis is unclear, particularly how TGFβ signaling is activated and then sustained through complex networks of cell-cell interaction not limited to the traditional fibrotic protagonist, the dermal fibroblast. Continued study of this rare disease will likely yield paradigms relevant to more common pathologies.</jats:p> |
| doi_str_mv | 10.3390/ijms22105104 |
| facet_avail | Online, Free |
| finc_class_facet | Chemie und Pharmazie, Physik, Informatik, Biologie |
| format | ElectronicArticle |
| format_de105 | Article, E-Article |
| format_de14 | Article, E-Article |
| format_de15 | Article, E-Article |
| format_de520 | Article, E-Article |
| format_de540 | Article, E-Article |
| format_dech1 | Article, E-Article |
| format_ded117 | Article, E-Article |
| format_degla1 | E-Article |
| format_del152 | Buch |
| format_del189 | Article, E-Article |
| format_dezi4 | Article |
| format_dezwi2 | Article, E-Article |
| format_finc | Article, E-Article |
| format_nrw | Article, E-Article |
| geogr_code | not assigned |
| geogr_code_person | not assigned |
| id | ai-49-aHR0cDovL2R4LmRvaS5vcmcvMTAuMzM5MC9pam1zMjIxMDUxMDQ |
| imprint | MDPI AG, 2021 |
| imprint_str_mv | MDPI AG, 2021 |
| institution | DE-Gla1, DE-Zi4, DE-15, DE-Rs1, DE-Pl11, DE-105, DE-14, DE-Ch1, DE-L229, DE-D275, DE-Bn3, DE-Brt1, DE-Zwi2, DE-D161 |
| issn | 1422-0067 |
| issn_str_mv | 1422-0067 |
| language | English |
| last_indexed | 2024-03-01T17:53:30.46Z |
| match_str | tartaglia2021impairedwoundhealingfibrosisandcancertheparadigmofrecessivedystrophicepidermolysisbullosa |
| mega_collection | MDPI AG (CrossRef) |
| physical | 5104 |
| publishDate | 2021 |
| publishDateSort | 2021 |
| publisher | MDPI AG |
| record_format | ai |
| recordtype | ai |
| series | International Journal of Molecular Sciences |
| source_id | 49 |
| spelling | Tartaglia, Grace Cao, Qingqing Padron, Zachary M. South, Andrew P. 1422-0067 MDPI AG Inorganic Chemistry Organic Chemistry Physical and Theoretical Chemistry Computer Science Applications Spectroscopy Molecular Biology General Medicine Catalysis http://dx.doi.org/10.3390/ijms22105104 <jats:p>Recessive Dystrophic Epidermolysis Bullosa (RDEB) is a devastating skin blistering disease caused by mutations in the gene encoding type VII collagen (C7), leading to epidermal fragility, trauma-induced blistering, and long term, hard-to-heal wounds. Fibrosis develops rapidly in RDEB skin and contributes to both chronic wounds, which emerge after cycles of repetitive wound and scar formation, and squamous cell carcinoma—the single biggest cause of death in this patient group. The molecular pathways disrupted in a broad spectrum of fibrotic disease are also disrupted in RDEB, and squamous cell carcinomas arising in RDEB are thus far molecularly indistinct from other sub-types of aggressive squamous cell carcinoma (SCC). Collectively these data demonstrate RDEB is a model for understanding the molecular basis of both fibrosis and rapidly developing aggressive cancer. A number of studies have shown that RDEB pathogenesis is driven by a radical change in extracellular matrix (ECM) composition and increased transforming growth factor-beta (TGFβ) signaling that is a direct result of C7 loss-of-function in dermal fibroblasts. However, the exact mechanism of how C7 loss results in extensive fibrosis is unclear, particularly how TGFβ signaling is activated and then sustained through complex networks of cell-cell interaction not limited to the traditional fibrotic protagonist, the dermal fibroblast. Continued study of this rare disease will likely yield paradigms relevant to more common pathologies.</jats:p> Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa International Journal of Molecular Sciences |
| spellingShingle | Tartaglia, Grace, Cao, Qingqing, Padron, Zachary M., South, Andrew P., International Journal of Molecular Sciences, Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa, Inorganic Chemistry, Organic Chemistry, Physical and Theoretical Chemistry, Computer Science Applications, Spectroscopy, Molecular Biology, General Medicine, Catalysis |
| title | Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa |
| title_full | Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa |
| title_fullStr | Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa |
| title_full_unstemmed | Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa |
| title_short | Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa |
| title_sort | impaired wound healing, fibrosis, and cancer: the paradigm of recessive dystrophic epidermolysis bullosa |
| title_unstemmed | Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa |
| topic | Inorganic Chemistry, Organic Chemistry, Physical and Theoretical Chemistry, Computer Science Applications, Spectroscopy, Molecular Biology, General Medicine, Catalysis |
| url | http://dx.doi.org/10.3390/ijms22105104 |