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Article

Functional Rescue of ABCC6 Deficiency by 4-Phenylbutyrate Therapy Reduces Dystrophic Calcification in Abcc6-/- Mice.

The Journal of investigative dermatology - 1 Mar 2017

Pomozi Viola, Brampton Christopher, Szeri Flóra, Dedinszki Dóra, Kozák Eszter, van de Wetering Koen, Hopkins Hi'ilani, Martin Ludovic, Váradi András, Le Saux Olivier

Abstract excerpt

Soft-tissue calcification is associated with aging, common conditions such as diabetes or hypercholesterolemia, and with certain genetic disorders. ABCC6 is an efflux transporter primarily expressed in liver facilitating the release of adenosine triphosphate from hepatocytes. Within the liver vasculature, adenosine triphosphate is converted into pyrophosphate, a major inhibitor of ectopic calcification. ABCC6...

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Identifiers

PMID: 27826008
DOI: 10.1016/j.jid.2016.10.035
PMCID: PMC5326614

Genes

ABCC6

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Functional Rescue of ABCC6 Deficiency by 4-Phenylbutyrate Therapy Reduces Dystrophic Calcification in Abcc6-/- Mice.PMID 27826008Read ↗

Pyrophosphate Supplementation Prevents Chronic and Acute Calcification in ABCC6-Deficient Mice.Semantic proximity Read ↗

Expression and in vivo rescue of human ABCC6 disease-causing mutants in mouse liver.Semantic proximity Read ↗

The molecular and physiological roles of ABCC6: more than meets the eyeSemantic proximity Read ↗

Analysis of pseudoxanthoma elasticum-causing missense mutants of ABCC6 in vivo; pharmacological correction of the mislocalized proteins.Semantic proximity Read ↗

Ectopic calcification in pseudoxanthoma elasticum responds to inhibition of tissue-nonspecific alkaline phosphatase.Semantic proximity Read ↗

Abcc6 Knockout Rat Model Highlights the Role of Liver in PPi Homeostasis in Pseudoxanthoma Elasticum.Semantic proximity Read ↗

Disruption of Abcc6 Transporter in Zebrafish Causes Ocular Calcification and Cardiac Fibrosis.Semantic proximity Read ↗

ABCC6 prevents ectopic mineralization seen in pseudoxanthoma elasticum by inducing cellular nucleotide release.Semantic proximity Read ↗

The ABCC6 Transporter: A New Player in Biomineralization.Semantic proximity Read ↗

High-dose oral pyrophosphate inhibits connective tissue calcification in Abcc6 null mice but affects bone structureSemantic proximity Read ↗

<i>Abcc6</i> null mice a model for mineralization disorder PXE show vertebral osteopenia without enhanced intervertebral disc calcification with agingSemantic proximity Read ↗

Inhibition of Tissue-Nonspecific Alkaline Phosphatase Attenuates Ectopic Mineralization in the Abcc6-/- Mouse Model of PXE but Not in the Enpp1 Mutant Mouse Models of GACI.Semantic proximity Read ↗

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