Evidence map›Paper›PMID 21435181›Full record

ArticleJournal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG2011

Pseudoxanthoma elasticum, the paradigm of heritable ectopic mineralization disorders - can diet help?

Jennifer LaRusso, Qiaoli Li, Jouni Uitto

Open access · greenAbstract read
In one paragraph

Article in Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.

0numbers the graph read from it
0cells of the map it votes in
7citing papers in PubMed
2.2field-weighted citation impact, top 12% 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

7 citing papers in PubMed, 16 citations in OpenAlex.

  1. Article
  2. Review
  3. Article
  4. [Pseudoxanthoma elasticum. More than a skin problem].Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete · 2013
    Article
  5. A case of pseudoxantoma elasticum.Journal of ocular biology, diseases, and informatics · 2012
    Article
  6. Article
  7. Pseudoxanthoma elasticum: report of two cases.Anais brasileiros de dermatologia
    Article
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

3 authors at 2 institutions in 1 country.

Jennifer LaRussoDepartment of Dermatology and Cutaneous Biology, Jefferson Medical College, Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, USA.
Qiaoli Li
Jouni Uitto
Thomas Jefferson University · USJefferson Institute · US

Funding

BIOCHEMISTRY AND MORPHOLOGY OF CONNECTIVE TISSUESR01AR028450 · NIAMS · THOMAS JEFFERSON UNIVERSITY · PI UITTO, JOUNI · 1986 to 2019
$6.5M
Model Systems for PXER01AR055225 · NIAMS · THOMAS JEFFERSON UNIVERSITY · PI UITTO, JOUNI · 2008 to 2017
$3.2M
NIAMS NIH HHS R01 AR028450NIAMS NIH HHS R01 AR055225
6 · The paper itself

Abstract

Pseudoxanthoma elasticum (PXE) is a heritable multi-system disorder manifesting with characteristic cutaneous lesions, associated with ocular findings and cardiovascular involvement. The skin lesions, yellowish papules which coalesce into plaques of inelastic and leathery skin, demonstrate by histopathologic and ultrastructural examinations ectopic mineralization of dermal connective tissues, primarily the elastic structures. PXE is inherited in an autosomal recessive fashion due to mutations in the ABCC6 gene. Significant insights into the pathogenesis of PXE have been recently obtained from observations on the Abcc6(-/-) knockout mouse which mimics the genetic, histopathologic and ultrastructural features of PXE. This mouse model has provided a platform to test various treatment modalities to counteract the mineralization phenotypes. One of the intriguing findings emanating from these studies is that supplementation of the mouse diet with magnesium, at levels that are ∼5-fold higher than those in control diet, completely inhibits the development of tissue mineralization. These and related observations suggest that changes in the diet might counteract the progression of PXE and improve the quality of life of patients with this, currently intractable, disease.

Indexed as

AnimalsATP-Binding Cassette, Sub-Family C ProteinsATP-Binding Cassette TransportersChromosome AberrationsDisease Models, AnimalDisease ProgressionDNA Mutational AnalysisDose-Response Relationship, DrugGenes, RecessiveHumansMagnesiumMicePhenotypePseudoxanthoma ElasticumQuality of LifeABCC6 protein, humanAbcc6 protein, mouseATP-Binding Cassette, Sub-Family C ProteinsATP-Binding Cassette TransportersMagnesium

Identifiers

PMID21435181
PMCPMC3307339
OpenAlexW2115180512

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.