Evidence map›Paper›PMID 38671506›Full record

ArticleSkeletal muscle2024

Pannexin 1 dysregulation in Duchenne muscular dystrophy and its exacerbation of dystrophic features in mdx mice.

Emily Freeman, Stéphanie Langlois, Marcos F Leyba, Tarek Ammar, Zacharie Léger, Hugh J McMillan, Jean-Marc Renaud, Bernard J Jasmin, Kyle N Cowan

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

Article in Skeletal muscle, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
0.7field-weighted citation impact, top 32% 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

1 citing paper in PubMed, 3 citations in OpenAlex.

  1. Article
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 3 institutions in 1 country.

Emily FreemanChildren's Hospital of Eastern Ontario Research Institute, Pediatric General Surgery, 401 Smyth Rd, Room 3360, Ottawa, ON, K1H 8L1, Canada.
Stéphanie LangloisDepartment of Surgery, Division of Pediatric Surgery, University of Ottawa, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.
Marcos F LeybaDepartment of Surgery, Division of Pediatric Surgery, University of Ottawa, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.
Tarek AmmarDepartment of Cellular and Molecular Medicine, University of Ottawa, Ottawa, ON, Canada.
Zacharie LégerChildren's Hospital of Eastern Ontario Research Institute, Pediatric General Surgery, 401 Smyth Rd, Room 3360, Ottawa, ON, K1H 8L1, Canada.
Hugh J McMillanChildren's Hospital of Eastern Ontario Research Institute, Pediatric General Surgery, 401 Smyth Rd, Room 3360, Ottawa, ON, K1H 8L1, Canada.
Jean-Marc RenaudDepartment of Cellular and Molecular Medicine, University of Ottawa, Ottawa, ON, Canada.
Bernard J JasminDepartment of Cellular and Molecular Medicine, University of Ottawa, Ottawa, ON, Canada.
Kyle N CowanDepartment of Surgery, Division of Pediatric Surgery, University of Ottawa, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada. kcowan@cheo.on.ca.
BC Centre for Disease Control · CAChildren's Hospital of Eastern Ontario · CAUniversity of Ottawa · CA

Funding

Association Française contre les Myopathies (AFM-Telethon) 23635CIHR Institute of Musculoskeletal Health and Arthritis (IMHA) MHM - 184012
6 · The paper itself

Abstract

backgroundDuchenne muscular dystrophy (DMD) is associated with impaired muscle regeneration, progressive muscle weakness, damage, and wasting. While the cause of DMD is an X-linked loss of function mutation in the gene encoding dystrophin, the exact mechanisms that perpetuate the disease progression are unknown. Our laboratory has demonstrated that pannexin 1 (Panx1 in rodents; PANX1 in humans) is critical for the development, strength, and regeneration of male skeletal muscle. In normal skeletal muscle, Panx1 is part of a multiprotein complex with dystrophin. We and others have previously shown that Panx1 levels and channel activity are dysregulated in various mouse models of DMD.

methodsWe utilized myoblast cell lines derived from DMD patients to assess PANX1 expression and function. To investigate how Panx1 dysregulation contributes to DMD, we generated a dystrophic (mdx) mouse model that lacks Panx1 (Panx1

resultsHere, we demonstrate that PANX1 levels and channel function are reduced in patient-derived DMD myoblast cell lines. Panx1

conclusionsCollectively, our findings suggest that PANX1/Panx1 dysregulation in DMD exacerbates several aspects of the disease. Moreover, our results suggest a potential therapeutic benefit to increasing PANX1 levels in dystrophic muscles.

Indexed as

ConnexinsMice, Inbred mdxMuscle, SkeletalMuscular Dystrophy, DuchenneNerve Tissue ProteinsAnimalsCell LineDisease Models, AnimalHumansMaleMiceMice, Inbred C57BLMice, KnockoutMuscle StrengthMyoblastsConnexinsNerve Tissue ProteinsPanx1 protein, mouseDuchenne muscular dystrophyMyoblastMyofiberPannexin 1Skeletal muscle

Identifiers

PMID38671506
PMCPMC11046831
OpenAlexW4395667356

What OpenQuestion holds

Textmetadata
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.