Evidence map›Paper›PMID 29079832›Full record

ArticleScientific reports2017

Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington's disease mice.

Marie K Bondulich, Nelly Jolinon, Georgina F Osborne, Edward J Smith, Ivan Rattray, Andreas Neueder, Kirupa Sathasivam, Mhoriam Ahmed, Nadira Ali, Agnesska C Benjamin and 19 more

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

Article in Scientific reports, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.

0numbers the graph read from it
0cells of the map it votes in
18citing papers in PubMed
1.3field-weighted citation impact, top 22% 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

18 citing papers in PubMed, 37 citations in OpenAlex.

  1. Trial
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  12. Article
  13. Molecular Mechanisms Underlying Muscle Wasting in Huntington's Disease.International journal of molecular sciences · 2020
    Review
  14. Article
  15. Article
  16. The first year.Acta neuropathologica · 2020
    Article
  17. Article
  18. Therapeutic and lifestyle approaches to obesity in older persons.Current opinion in clinical nutrition and metabolic care · 2019
    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

29 authors at 6 institutions in 2 countries.

Marie K BondulichSobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, WC1N 3BG, UK.
Nelly JolinonDepartment Medical and Molecular Genetics, King's College London, London, SE1 9RT, UK.
Georgina F OsborneSobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, WC1N 3BG, UK.
Edward J SmithSobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, WC1N 3BG, UK.
Ivan RattrayDepartment Medical and Molecular Genetics, King's College London, London, SE1 9RT, UK.
Andreas NeuederSobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, WC1N 3BG, UK.
Kirupa SathasivamSobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, WC1N 3BG, UK.
Mhoriam AhmedSobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, WC1N 3BG, UK.
Nadira AliSobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, WC1N 3BG, UK.
Agnesska C BenjaminSobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, WC1N 3BG, UK.
Xiaoli ChangDepartment Molecular Biology and Genetics, The Johns Hopkins University School of Medicine, Baltimore, MD, 21205, USA.
James R T DickSobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, WC1N 3BG, UK.
Matthew EllisDivision of Neuropathology, UCL Institute of Neurology, London, WC1N 3BG, UK.
Sophie A FranklinSobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, WC1N 3BG, UK.
Daniel GoodwinSobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, WC1N 3BG, UK.
Linda InuabasiDepartment Medical and Molecular Genetics, King's College London, London, SE1 9RT, UK.
Hayley LazellSobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, WC1N 3BG, UK.
Adam LeharDepartment Molecular Biology and Genetics, The Johns Hopkins University School of Medicine, Baltimore, MD, 21205, USA.
Angela Richard-LondtDivision of Neuropathology, UCL Institute of Neurology, London, WC1N 3BG, UK.
Jim RosinskiCHDI Management/CHDI Foundation Inc, New York, NY, 10001, USA.
Donna L SmithDepartment Medical and Molecular Genetics, King's College London, London, SE1 9RT, UK.
Tobias WoodDepartment of Neuroimaging, King's College London, Institute of Psychiatry, London, SE5 8AF, UK.
Sarah J TabriziHuntington's Disease Centre, UCL Institute of Neurology, London, WC1N 3BG, UK.
Sebastian BrandnerDivision of Neuropathology, UCL Institute of Neurology, London, WC1N 3BG, UK.ORCID 0000-0002-9821-0342
Linda GreensmithSobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, WC1N 3BG, UK.
David HowlandCHDI Management/CHDI Foundation Inc, New York, NY, 10001, USA.
Ignacio Munoz-SanjuanCHDI Management/CHDI Foundation Inc, New York, NY, 10001, USA.
Se-Jin LeeDepartment Molecular Biology and Genetics, The Johns Hopkins University School of Medicine, Baltimore, MD, 21205, USA.
Gillian P BatesSobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, WC1N 3BG, UK. gillian.bates@ucl.ac.uk.ORCID 0000-0002-4041-6305
King's College London · GBCHDI Foundation · USInstitute for Neurodegenerative Disorders · USJohns Hopkins University · USUniversity College London · GBHuntington's Disease Association · GB

Funding

Characterization of Myostatin and GDF-11R01AR060636 · NIAMS · UNIVERSITY OF CONNECTICUT SCH OF MED/DNT · PI LEE, SE-JIN · 2010 to 2020
$3.7M
Department of HealthMedical Research Council MR/L003627/1Medical Research Council MR/L003627/2Medical Research Council MR/L012936/1Medical Research Council MR/L02053X/1Medical Research Council MR/P007015/1NIAMS NIH HHS R01 AR060636Wellcome Trust 200181/Z/15/Z
6 · The paper itself

Abstract

Huntington's disease (HD) is an inherited neurodegenerative disorder of which skeletal muscle atrophy is a common feature, and multiple lines of evidence support a muscle-based pathophysiology in HD mouse models. Inhibition of myostatin signaling increases muscle mass, and therapeutic approaches based on this are in clinical development. We have used a soluble ActRIIB decoy receptor (ACVR2B/Fc) to test the effects of myostatin/activin A inhibition in the R6/2 mouse model of HD. Weekly administration from 5 to 11 weeks of age prevented body weight loss, skeletal muscle atrophy, muscle weakness, contractile abnormalities, the loss of functional motor units in EDL muscles and delayed end-stage disease. Inhibition of myostatin/activin A signaling activated transcriptional profiles to increase muscle mass in wild type and R6/2 mice but did little to modulate the extensive Huntington's disease-associated transcriptional dysregulation, consistent with treatment having little impact on HTT aggregation levels. Modalities that inhibit myostatin signaling are currently in clinical trials for a variety of indications, the outcomes of which will present the opportunity to assess the potential benefits of targeting this pathway in HD patients.

Indexed as

Activin Receptors, Type IIAnimalsBody WeightHand StrengthHuntingtin ProteinHuntington DiseaseMaleMiceMuscle, SkeletalMuscular AtrophyMyostatinProtein AggregatesActivin Receptors, Type IIHuntingtin ProteinMyostatinProtein Aggregates

Identifiers

PMID29079832
PMCPMC5660167
OpenAlexW2766503299

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.