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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...
Autores principales: | Tiemann, Janina, Wagner, Thomas, Lindenkamp, Christopher, Plümers, Ricarda, Faust, Isabel, Knabbe, Cornelius, Hendig, Doris |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2020
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Materias: | |
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 |
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