Linking ABCC6 Deficiency in Primary Human Dermal Fibroblasts of PXE Patients to p21-Mediated Premature Cellular Senescence and the Development of a Proinflammatory Secretory Phenotype

Pseudoxanthoma elasticum (PXE) is a rare autosomal-recessive disorder that is mainly caused by mutations in the ATP-binding cassette sub-family C member 6 (ABCC6) gene. Clinically PXE is characterized by a loss of skin elasticity, arteriosclerosis or visual impairments. It also shares some molecular...

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Detalles Bibliográficos
Autores principales: Tiemann, Janina, Wagner, Thomas, Lindenkamp, Christopher, Plümers, Ricarda, Faust, Isabel, Knabbe, Cornelius, Hendig, Doris
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7766446/
https://www.ncbi.nlm.nih.gov/pubmed/33352936
http://dx.doi.org/10.3390/ijms21249665

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7766446/
https://www.ncbi.nlm.nih.gov/pubmed/33352936
http://dx.doi.org/10.3390/ijms21249665

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