Evidence map›Paper›PMID 22163031›Full record

ArticlePloS one2011

Alkyl-glycerol rescues plasmalogen levels and pathology of ether-phospholipid deficient mice.

Pedro Brites, Ana Sofia Ferreira, Tiago Ferreira da Silva, Vera F Sousa, Ana R Malheiro, Marinus Duran, Hans R Waterham, Myriam Baes, Ronald J A Wanders

Open access · goldAbstract read
In one paragraph

Article in PloS one, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 61 papers.

0numbers the graph read from it
0cells of the map it votes in
61citing papers in PubMed
3.6field-weighted citation impact, top 6% 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

61 citing papers in PubMed, 120 citations in OpenAlex.

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1 more citing papers are in PubMed but not listed here.

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

9 authors at 5 institutions in 4 countries.

Pedro BritesNerve Regeneration Group, Instituto de Biologia Molecular e Celular, Porto, Portugal. pedro.brites@ibmc.up.pt
Ana Sofia Ferreira
Tiago Ferreira da Silva
Vera F Sousa
Ana R Malheiro
Marinus Duran
Hans R Waterham
Myriam Baes
Ronald J A Wanders
Instituto de Biologia Molecular e CelularAcademic Medical Center · NLUniversity of Amsterdam · NLKU Leuven · BEUniversidade do Porto · PT

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

A deficiency of plasmalogens, caused by impaired peroxisomal metabolism affects normal development and multiple organs in adulthood. Treatment options aimed at restoring plasmalogen levels may be relevant for the therapy of peroxisomal and non-peroxisomal disorders. In this study we determined the in vivo efficacy of an alkyl glycerol (AG), namely, 1-O-octadecyl-rac-glycerol, as a therapeutic agent for defects in plasmalogen synthesis. To achieve this, Pex7 knockout mice, a mouse model for Rhizomelic Chondrodysplasia Punctata type 1 characterized by the absence of plasmalogens, and WT mice were fed a control diet or a diet containing 2% alkyl-glycerol. Plasmalogen levels were measured in target organs and the biochemical data were correlated with the histological analysis of affected organs. Plasmalogen levels in all peripheral tissues of Pex7 KO mice fed the AG diet for 2 months normalized to the levels of AG fed WT mice. In nervous tissues of Pex7 KO mice fed the AG-diet, plasmalogen levels were significantly increased compared to control fed KO mice. Histological analysis of target organs revealed that the AG-diet was able to stop the progression of the pathology in testis, adipose tissue and the Harderian gland. Interestingly, the latter tissues are characterized by the presence of lipid droplets which were absent or reduced in size and number when ether-phospholipids are lacking, but which can be restored with the AAG treatment. Furthermore, nerve conduction in peripheral nerves was improved. When given prior to the occurrence of major pathological changes, the AG-diet prevented or ameliorated the pathology observed in Pex7 KO mice depending on the degree of plasmalogen restoration. This study provides evidence of the beneficial effects of treating a plasmalogen deficiency with alkyl-glycerol.

Indexed as

Animal FeedAnimalsCell LineElectrophysiologyGenotypeGlycerolLipidsMiceMice, KnockoutNerve TissueNeural ConductionPeroxisomal Targeting Signal 2 ReceptorPhospholipid EthersPhospholipidsPlasmalogensReceptors, Cytoplasmic and NuclearGlycerolLipidsPeroxisomal Targeting Signal 2 ReceptorPhospholipid EthersPhospholipidsPlasmalogensReceptors, Cytoplasmic and Nuclear

Identifiers

PMID22163031
PMCPMC3232224
OpenAlexW2051287539

What OpenQuestion holds

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LicenceCC BY
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.