Evidence map›Paper›PMID 28416300›Full record

ArticleThe American journal of pathology2017

Pyrophosphate Supplementation Prevents Chronic and Acute Calcification in ABCC6-Deficient Mice.

Viola Pomozi, Christopher Brampton, Koen van de Wetering, Janna Zoll, Bianca Calio, Kevin Pham, Jesse B Owens, Joel Marh, Stefan Moisyadi, András Váradi and 5 more

Registry-linked trialOpen access · bronzeAbstract read
In one paragraph

Article in The American journal of pathology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT03813550 (Intestinal Microbiota and Vitamin K Levels in PXE Patients), which is not on this map. Cited by 60 papers.

0numbers the graph read from it
0cells of the map it votes in
60citing papers in PubMed
6.4field-weighted citation impact, top 4% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT03813550 naunknown statusnot on this mapstarted 2019, after this paper: background citation

Intestinal Microbiota and Vitamin K Levels in PXE Patients (IMPROVE Study)

TypeinterventionalSponsorUniversity Hospital, AngersRan2019 to 2020Enrolled20ConditionsPseudoxanthoma ElasticumArmsFecal and blood samples
3 · Its place in the literature

Who cites it

60 citing papers in PubMed, 78 citations in OpenAlex.

  1. Review
  2. Article
  3. Article
  4. Review
  5. Soft tissue calcifications in chronic kidney disease-beyond the vasculature.Pflugers Archiv : European journal of physiology · 2025
    Review
  6. Review
  7. Article
  8. Article
  9. Review
  10. Article
  11. Novel treatment for PXE: Recombinant ENPP1 enzyme therapy.Molecular therapy : the journal of the American Society of Gene Therapy · 2024
    Article
  12. Oral pyrophosphate protects Abcc6Journal of molecular medicine (Berlin, Germany) · 2024
    Article
  13. Review
  14. Article
  15. A Plasma Pyrophosphate Cutoff Value for Diagnosing Pseudoxanthoma Elasticum.International journal of molecular sciences · 2024
    Article
  16. Review
  17. Review
  18. Article
  19. Review
  20. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

15 authors at 5 institutions in 4 countries.

Viola PomoziDepartment of Cell and Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, Hawaii.
Christopher BramptonDepartment of Cell and Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, Hawaii.
Koen van de WeteringDepartment of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, PXE International Center of Excellence in Research and Clinical Care, Thomas Jefferson University, Philadelphia, Pennsylvania.
Janna ZollDepartment of Cell and Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, Hawaii.
Bianca CalioDepartment of Cell and Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, Hawaii.
Kevin PhamDepartment of Cell and Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, Hawaii.
Jesse B OwensInstitute for Biogenesis Research, John A. Burns School of Medicine, University of Hawaii, Honolulu, Hawaii.
Joel MarhInstitute for Biogenesis Research, John A. Burns School of Medicine, University of Hawaii, Honolulu, Hawaii.
Stefan MoisyadiInstitute for Biogenesis Research, John A. Burns School of Medicine, University of Hawaii, Honolulu, Hawaii.
András VáradiInstitute of Enzymology, Research Centre for Natural Sciences, Hungarian Academy of Sciences, Budapest, Hungary.
Ludovic MartinUniversité Bretagne-Loire, Integrated Neurovascular and Mitochondrial Biology, National Center for Scientific Research 6214/INSERM 1083, Angers, France; University Hospital Angers, Center for PXE Consultation, Angers, France.
Carolin BauerInstitut für Integrative und Experimentelle Genomik Universität zu Lübeck, German Centre for Cardiovascular Research, Partner Site Hamburg/Kiel/Lübeck, Germany; University Heart Centre Lübeck, Universität zu Lübeck, Lübeck, Germany.
Jeanette ErdmannInstitut für Integrative und Experimentelle Genomik Universität zu Lübeck, German Centre for Cardiovascular Research, Partner Site Hamburg/Kiel/Lübeck, Germany; University Heart Centre Lübeck, Universität zu Lübeck, Lübeck, Germany.
Zouhair AherrahrouInstitut für Integrative und Experimentelle Genomik Universität zu Lübeck, German Centre for Cardiovascular Research, Partner Site Hamburg/Kiel/Lübeck, Germany; University Heart Centre Lübeck, Universität zu Lübeck, Lübeck, Germany.
Olivier Le SauxDepartment of Cell and Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, Hawaii. Electronic address: lesaux@hawaii.edu.
University of Hawaiʻi at Mānoa · USUniversität Hamburg · DEInserm · FRInstitute of Molecular Life Sciences · HUThomas Jefferson University · US

Funding

UH Hilo COP A&RP20GM103466 · NIGMS · UNIVERSITY OF HAWAII AT MANOA · PI Peter R Hoffmann · 2012 to 2026
$60.3M
WORLDWIDE EPIDEMIOLOGY &EPIZOOTIOLOGY OF HANTAVIRUSG12RR003061 · NCRR · UNIVERSITY OF HAWAII AT MANOA · PI COUVILLON, PATRICIA A · 1986 to 2011
$32.3M
RESEARCH DESIGN, BIOSTATISTICS AND RESEARCH ETHICS COREG12MD007601 · NIMHD · UNIVERSITY OF HAWAII AT MANOA · PI BERRY, MARLA J · 2012 to 2016
$13.0M
Transgenic Mouse, ICSI and IVF CoreP20GM103457 · NIGMS · UNIVERSITY OF HAWAII AT MANOA · PI WARD, WILLIAM S · 2012 to 2018
$12.9M
Pilot Project ProgramP30GM103341 · NIGMS · UNIVERSITY OF HAWAII AT MANOA · PI SHOHET, RALPH V. · 2012 to 2016
$5.5M
The role of ABCC6 in chronic and acute cardiovascular mineralizationR01HL108249 · NHLBI · UNIVERSITY OF HAWAII AT MANOA · PI BOISVERT, WILLIAM A, LE SAUX, OLIVIER · 2012 to 2015
$1.4M
NCRR NIH HHS G12 RR003061NHLBI NIH HHS R01 HL108249NIGMS NIH HHS P20 GM103457NIGMS NIH HHS P20 GM103466NIGMS NIH HHS P30 GM103341NIMHD NIH HHS G12 MD007601
6 · The paper itself

Abstract

Soft tissue calcification occurs in several common acquired pathologies, such as diabetes and hypercholesterolemia, or can result from genetic disorders. ABCC6, a transmembrane transporter primarily expressed in liver and kidneys, initiates a molecular pathway inhibiting ectopic calcification. ABCC6 facilitates the cellular efflux of ATP, which is rapidly converted into pyrophosphate (PPi), a major calcification inhibitor. Heritable mutations in ABCC6 underlie the incurable calcification disorder pseudoxanthoma elasticum and some cases of generalized arterial calcification of infancy. Herein, we determined that the administration of PPi and the bisphosphonate etidronate to Abcc6

Indexed as

Acute DiseaseAnimalsATP-Binding Cassette, Sub-Family C ProteinsATP-Binding Cassette TransportersCalcinosisChronic DiseaseDiphosphatesDisease Models, AnimalDose-Response Relationship, DrugDrug Evaluation, PreclinicalEtidronic AcidFemaleLiverMaleMice, Inbred C57BLMice, KnockoutABCC6 protein, humanAbcc6 protein, mouseATP-Binding Cassette, Sub-Family C ProteinsATP-Binding Cassette TransportersDiphosphatesdiphosphoric acidEtidronic Acid

Identifiers

PMID28416300
PMCPMC5455066
OpenAlexW2607299292

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.