Evidence map›Paper›PMID 35511611›Full record

ArticleExperimental dermatology2022

INZ-701, a recombinant ENPP1 enzyme, prevents ectopic calcification in an Abcc6

Ida Joely Jacobs, Zhiliang Cheng, Douglas Ralph, Kevin O'Brien, Lisa Flaman, Jennifer Howe, David Thompson, Jouni Uitto, Qiaoli Li, Yves Sabbagh

Open access · greenAbstract read
In one paragraph

Article in Experimental dermatology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.

0numbers the graph read from it
0cells of the map it votes in
20citing papers in PubMed
3.4field-weighted citation impact, top 7% 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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

20 citing papers in PubMed, 23 citations in OpenAlex.

  1. Trial
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  8. Article
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  11. Novel treatment for PXE: Recombinant ENPP1 enzyme therapy.Molecular therapy : the journal of the American Society of Gene Therapy · 2024
    Article
  12. Article
  13. Article
  14. Review
  15. Review
  16. Review
  17. Weighing the Evidence for the Roles of Plasma Versus Local Pyrophosphate in Ectopic Calcification Disorders.Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research · 2023
    Article
  18. High Bone Mass Disorders: New Insights From Connecting the Clinic and the Bench.Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research · 2023
    Review
  19. Article
  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

10 authors at 2 institutions in 2 countries.

Ida Joely JacobsDepartment of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
Zhiliang ChengInozyme Pharma, Boston, Massachusetts, USA.
Douglas RalphDepartment of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.ORCID 0000-0003-2910-1342
Kevin O'BrienInozyme Pharma, Boston, Massachusetts, USA.
Lisa FlamanInozyme Pharma, Boston, Massachusetts, USA.
Jennifer HoweInozyme Pharma, Boston, Massachusetts, USA.
David ThompsonInozyme Pharma, Boston, Massachusetts, USA.
Jouni UittoDepartment of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.ORCID 0000-0003-4639-807X
Qiaoli LiDepartment of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.ORCID 0000-0002-8495-7103
Yves SabbaghInozyme Pharma, Boston, Massachusetts, USA.
Novozymes (Denmark) · DKThomas Jefferson University · US

Funding

Novel Treatments for PXER01AR072695 · NIAMS · THOMAS JEFFERSON UNIVERSITY · PI LI, QIAOLI, VAN DE WETERING, KOEN · 2018 to 2022
$1.7M
Pharmacologic Intervention for Ectopic CalcificationR21AR077332 · NIAMS · THOMAS JEFFERSON UNIVERSITY · PI LI, QIAOLI · 2021 to 2022
$392k
NIAMS NIH HHS R01 AR072695NIAMS NIH HHS R21 AR077332
6 · The paper itself

Abstract

Pseudoxanthoma elasticum (PXE), a heritable multisystem ectopic calcification disorder, is predominantly caused by inactivating mutations in ABCC6. The encoded protein, ABCC6, is a hepatic efflux transporter and a key regulator of extracellular inorganic pyrophosphate (PPi). Recent studies demonstrated that deficiency of plasma PPi, a potent endogenous calcification inhibitor, is the underlying cause of PXE. This study examined whether restoring plasma PPi levels by INZ-701, a recombinant human ENPP1 protein, the principal PPi-generating enzyme, prevents ectopic calcification in an Abcc6

Indexed as

CalcinosisPhosphoric Diester HydrolasesPseudoxanthoma ElasticumPyrophosphatasesAnimalsATP-Binding Cassette, Sub-Family C ProteinsDisease Models, AnimalHumansLiverMiceMice, KnockoutRecombinant ProteinsSkinAbcc6 protein, mouseATP-Binding Cassette, Sub-Family C Proteinsectonucleotide pyrophosphatase phosphodiesterase 1Phosphoric Diester HydrolasesPyrophosphatasesRecombinant Proteinsanimal modelectopic calcificationinorganic pyrophosphateINZ-701pseudoxanthoma elasticum

Identifiers

PMID35511611
PMCPMC10077110
OpenAlexW4229054505

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

Registered trials

None linked

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.