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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Dettagli Bibliografici
Pubblicato in:J Invest Dermatol
Autori principali: Pomozi, Viola, Julian, Charnelle B., Zoll, Janna, Pham, Kevin, Kuo, Sheree, Tőkési, Natália, Martin, Ludovic, Váradi, András, Le Saux, Olivier
Natura: Artigo
Lingua:Inglês
Pubblicazione: 2018
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Article
Accesso online: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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