Evidence map›Paper›PMID 26900448›Full record

ArticleSkeletal muscle2016

Prenatal muscle development in a mouse model for the secondary dystroglycanopathies.

Jihee Kim, Mark Hopkinson, Manoli Kavishwar, Marta Fernandez-Fuente, Susan Carol Brown

Open access · goldAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Skeletal muscle, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
0.5field-weighted citation impact, top 33% 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

4 citing papers in PubMed, 6 citations in OpenAlex.

  1. Review
  2. Review
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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

5 authors at 2 institutions in 1 country.

Jihee KimDepartment of Comparative Biomedical Sciences, Royal Veterinary College, University of London, London, UK.
Mark HopkinsonDepartment of Comparative Biomedical Sciences, Royal Veterinary College, University of London, London, UK.
Manoli KavishwarDepartment of Comparative Biomedical Sciences, Royal Veterinary College, University of London, London, UK.
Marta Fernandez-FuenteDepartment of Comparative Biomedical Sciences, Royal Veterinary College, University of London, London, UK.
Susan Carol BrownDepartment of Comparative Biomedical Sciences, Royal Veterinary College, University of London, London, UK.
University of London · GBRoyal Veterinary College · GB

Funding

Medical Research Council G0200171
6 · The paper itself

Abstract

backgroundThe defective glycosylation of α-dystroglycan is associated with a group of muscular dystrophies that are collectively referred to as the secondary dystroglycanopathies. Mutations in the gene encoding fukutin-related protein (FKRP) are one of the most common causes of secondary dystroglycanopathy in the UK and are associated with a wide spectrum of disease. Whilst central nervous system involvement has a prenatal onset, no studies have addressed prenatal muscle development in any of the mouse models for this group of diseases. In view of the pivotal role of α-dystroglycan in early basement membrane formation, we sought to determine if the muscle formation was altered in a mouse model of FKRP-related dystrophy.

resultsMice with a knock-down in FKRP (FKRP(KD)) showed a marked reduction in α-dystroglycan glycosylation and reduction in laminin binding by embryonic day 15.5 (E15.5), relative to wild type controls. In addition, the total number of Pax7(+) progenitor cells in the FKRP(KD) tibialis anterior at E15.5 was significantly reduced, and myotube cluster/myofibre size showed a significant reduction in size. Moreover, myoblasts isolated from the limb muscle of these mice at E15.5 showed a marked reduction in their ability to form myotubes in vitro.

conclusionsThese data identify an early reduction of laminin α2, reduction of myogenicity and depletion of Pax7(+) progenitor cells which would be expected to compromise subsequent postnatal muscle growth and its ability to regenerate postnatally. These findings are of significance to the development of future therapies in this group of devastating conditions.

Indexed as

Muscle DevelopmentAnimalsCells, CulturedDisease Models, AnimalDystroglycansGenetic Predisposition to DiseaseGestational AgeGlycosylationLamininMice, KnockoutMuscle Fibers, SkeletalMuscle, SkeletalMyoblasts, SkeletalPAX7 Transcription FactorPentosyltransferasesPhenotypeDystroglycansFkrp protein, mouseLamininlaminin alpha 2Pax7 protein, mousePAX7 Transcription FactorPentosyltransferasesProteinsTransferasesCongenital muscular dystrophyDystroglycanDystroglycanopathyFukutin-related proteinLamininPax7

Identifiers

PMID26900448
PMCPMC4759920
OpenAlexW2282507688

What OpenQuestion holds

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LicenceCC BY
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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.