DIETARY PYROPHOSPHATE MODULATES CALCIFICATION IN A MOUSE MODEL OF PSEUDOXANTHOMA ELASTICUM: IMPLICATION FOR TREATMENT OF PATIENTS

Pseudoxanthoma elasticum (PXE) is a heritable disease caused by ABCC6 deficiency. Patients develop ectopic calcification in skin, eyes and vascular tissues. ABCC6, primarily found in liver and kidneys, mediates the cellular efflux of ATP, which is rapidly converted into pyrophosphate (PPi), a potent...

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Detalhes bibliográficos
Publicado no:J Invest Dermatol
Main Authors: Pomozi, Viola, Julian, Charnelle B., Zoll, Janna, Pham, Kevin, Kuo, Sheree, Tőkési, Natália, Martin, Ludovic, Váradi, András, Le Saux, Olivier
Formato: Artigo
Idioma:Inglês
Publicado em: 2018
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6478508/
https://ncbi.nlm.nih.gov/pubmed/30468740
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.jid.2018.10.040
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