Evidence map›Paper›PMID 39518930›Full record

ArticleInternational journal of molecular sciences2024

Molecular, Histological, and Functional Changes in Acta1-MCM;FLExDUX4/+ Mice.

Solene Sohn, Sophie Reid, Maximilien Bowen, Emilio Corbex, Laura Le Gall, Eva Sidlauskaite, Christophe Hourde, Baptiste Morel, Virginie Mariot, Julie Dumonceaux

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in International journal of molecular sciences, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
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

2 citing papers in PubMed.

  1. Article
  2. Veterinary world · 2025
    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.

Solene SohnNIHR Biomedical Research Centre, University College London, Great Ormond Street Institute of Child Health and Great Ormond Street Hospital NHS Trust, London WC1N 1EH, UK.ORCID 0000-0001-9558-2485
Sophie ReidNIHR Biomedical Research Centre, University College London, Great Ormond Street Institute of Child Health and Great Ormond Street Hospital NHS Trust, London WC1N 1EH, UK.ORCID 0009-0008-2722-8220
Maximilien BowenLaboratoire Interuniversitaire de Biologie de la Motricité LIBM, EA 7424, Savoie Mont Blanc University, F-7300 Chambéry, France.
Emilio CorbexNIHR Biomedical Research Centre, University College London, Great Ormond Street Institute of Child Health and Great Ormond Street Hospital NHS Trust, London WC1N 1EH, UK.
Laura Le GallNIHR Biomedical Research Centre, University College London, Great Ormond Street Institute of Child Health and Great Ormond Street Hospital NHS Trust, London WC1N 1EH, UK.
Eva SidlauskaiteNIHR Biomedical Research Centre, University College London, Great Ormond Street Institute of Child Health and Great Ormond Street Hospital NHS Trust, London WC1N 1EH, UK.
Christophe HourdeLaboratoire Interuniversitaire de Biologie de la Motricité LIBM, EA 7424, Savoie Mont Blanc University, F-7300 Chambéry, France.
Baptiste MorelLaboratoire Interuniversitaire de Biologie de la Motricité LIBM, EA 7424, Savoie Mont Blanc University, F-7300 Chambéry, France.ORCID 0000-0002-2872-6423
Virginie MariotNIHR Biomedical Research Centre, University College London, Great Ormond Street Institute of Child Health and Great Ormond Street Hospital NHS Trust, London WC1N 1EH, UK.ORCID 0000-0002-3222-9286
Julie DumonceauxNIHR Biomedical Research Centre, University College London, Great Ormond Street Institute of Child Health and Great Ormond Street Hospital NHS Trust, London WC1N 1EH, UK.ORCID 0000-0002-2052-9361

Funding

AFM 22582French National Research Agency ANR-22-CE14-0073
6 · The paper itself

Abstract

DUX4 is the major gene responsible for facioscapulohumeral dystrophy (FSHD). Several mouse models expressing DUX4 have been developed, the most commonly used by academic laboratories being ACTA1-MCM/FLExDUX4. In this study, molecular and histological modifications in the tibialis anterior and quadriceps muscles were investigated in this model at different time points. We investigated several changes that could be used as markers of therapeutic efficacy. Our results confirm the progressive muscular dystrophy previously described but also highlight biases associated with tamoxifen injections and the complexity of choosing the genes used to calculate a DUX4-pathway gene composite score. We also developed a comprehensive force test that better reflects the movements made in everyday life. This functional force-velocity-endurance model, which describes the force production capacities at all velocity and fatigue levels, was applied on 12-13-week-old animals without tamoxifen. Our data highlight that previously unsuspected muscle properties are also affected by the expression of DUX4, leading to a weaker muscle with a lower initial muscle force but with preserved power and endurance capacity. Importantly, this force-velocity-endurance approach can be used in humans for clinical evaluations.

Indexed as

Homeodomain ProteinsMuscle, SkeletalActinsAnimalsDisease Models, AnimalMaleMiceMice, TransgenicMuscular Dystrophy, FacioscapulohumeralTamoxifenActinsDux4 protein, mouseHomeodomain ProteinsTamoxifenbiomarkerDUX4force testFSHDmouse modelmusclemyopathystrength

Identifiers

PMID39518930
PMCPMC11545788

What OpenQuestion holds

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Registered trials

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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.