Evidence map›Paper›PMID 28823869›Full record

ArticleThe American journal of pathology2017

Intermittent Glucocorticoid Dosing Improves Muscle Repair and Function in Mice with Limb-Girdle Muscular Dystrophy.

Mattia Quattrocelli, Isabella M Salamone, Patrick G Page, James L Warner, Alexis R Demonbreun, Elizabeth M McNally

Erratum issued Registry-linked trialOpen access · bronzeAbstract read
In one paragraph

Article in The American journal of pathology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. It is linked to trial NCT04054375 (Open Label Safety and Efficacy of Once Weekly Steroid in Patients With LGMD and Becker Muscular Dystrophy), which is not on this map. Cited by 33 papers.

0numbers the graph read from it
0cells of the map it votes in
33citing papers in PubMed
2.1field-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.

NCT04054375 phase2completednot on this mapstarted 2019, after this paper: background citation

Open Label Safety and Efficacy of Once Weekly Steroid in Patients With LGMD and Becker Muscular Dystrophy

TypeinterventionalSponsorNorthwestern UniversityRan2019 to 2022Enrolled20ConditionsLimb-girdle Muscular Dystrophy, Becker Muscular DystrophyArmsPrednisone
3 · Its place in the literature

Who cites it

33 citing papers in PubMed, 47 citations in OpenAlex.

  1. Trial
  2. Review
  3. Review
  4. Article
  5. Article
  6. Uncovering compound heterozygousFrontiers in genetics · 2025
    Article
  7. Article
  8. Article
  9. Article
  10. Molecular mechanisms and therapeutic strategies for neuromuscular diseases.Cellular and molecular life sciences : CMLS · 2024
    Review
  11. Review
  12. Article
  13. Article
  14. Article
  15. Article
  16. Article
  17. Review
  18. Article
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4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

6 authors at 1 institution in 1 country.

Mattia QuattrocelliCenter for Genetic Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Isabella M SalamoneCenter for Genetic Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Patrick G PageCenter for Genetic Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
James L WarnerCenter for Genetic Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Alexis R DemonbreunCenter for Genetic Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Elizabeth M McNallyCenter for Genetic Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Electronic address: elizabeth.mcnally@northwestern.edu.
Northwestern University · US

Funding

Tumor Environment and Metastasis (TEAM) Research ProgramP30CA060553 · NCI · NORTHWESTERN UNIVERSITY AT CHICAGO · PI LEONIDAS C. PLATANIAS · 1993 to 2026
$153.9M
Training and education CoreU54AR052646 · NIAMS · UNIVERSITY OF PENNSYLVANIA · PI MCNALLY, ELIZABETH M, SWEENEY, H LEE · 2005 to 2020
$24.1M
SARCOGLYCAN IN MYOPATHY AND MUSCLE MEMBRANE STABILITYR01HL061322 · NHLBI · UNIVERSITY OF CHICAGO · PI MCNALLY, ELIZABETH M · 1999 to 2022
$7.5M
Myoferlin in Muscle Membrane Fusion and RepairR01NS047726 · NINDS · UNIVERSITY OF CHICAGO · PI ALEXIS R. DEMONBREUN, Elizabeth M McNally · 2003 to 2026
$7.2M
NCI NIH HHS P30 CA060553NHLBI NIH HHS R01 HL061322NIAMS NIH HHS U54 AR052646NINDS NIH HHS R01 NS047726
6 · The paper itself

Abstract

The muscular dystrophies are genetically diverse. Shared pathological features among muscular dystrophies include breakdown, or loss of muscle, and accompanying fibrotic replacement. Novel strategies are needed to enhance muscle repair and function and to slow this pathological remodeling. Glucocorticoid steroids, like prednisone, are known to delay loss of ambulation in patients with Duchenne muscular dystrophy but are accompanied by prominent adverse effects. However, less is known about the effects of steroid administration in other types of muscular dystrophies, including limb-girdle muscular dystrophies (LGMDs). LGMD 2B is caused by loss of dysferlin, a membrane repair protein, and LGMD 2C is caused by loss of the dystrophin-associated protein, γ-sarcoglycan. Herein, we assessed the efficacy of steroid dosing on sarcolemmal repair, muscle function, histopathology, and the regenerative capacity of primary muscle cells. We found that in murine models of LGMD 2B and 2C, daily prednisone dosing reduced muscle damage and fibroinflammatory infiltration. However, daily prednisone dosing also correlated with increased muscle adipogenesis and atrophic remodeling. Conversely, intermittent dosing of prednisone, provided once weekly, enhanced muscle repair and did not induce atrophy or adipogenesis, and was associated with improved muscle function. These data indicate that dosing frequency of glucocorticoid steroids affects muscle remodeling in non-Duchenne muscular dystrophies, suggesting a positive outcome associated with intermittent steroid dosing in LGMD 2B and 2C muscle.

Indexed as

AnimalsDystrophinGlucocorticoidsMembrane ProteinsMiceMuscle, SkeletalMuscular Dystrophies, Limb-GirdleMuscular Dystrophy, DuchennePrednisoneSarcoglycansDystrophinGlucocorticoidsMembrane ProteinsPrednisoneSarcoglycans

Identifiers

PMID28823869
PMCPMC5809598
OpenAlexW2745781290

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

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.