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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...

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Detalhes bibliográficos
Publicado no:Int J Mol Sci
Main Authors: Tiemann, Janina, Wagner, Thomas, Lindenkamp, Christopher, Plümers, Ricarda, Faust, Isabel, Knabbe, Cornelius, Hendig, Doris
Formato: Artigo
Idioma:Inglês
Publicado em: MDPI 2020
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7766446/
https://ncbi.nlm.nih.gov/pubmed/33352936
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3390/ijms21249665
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