Evidence map›Paper›PMID 34796900›Full record

ArticleDisease models & mechanisms2021

A scalable, clinically severe pig model for Duchenne muscular dystrophy.

Michael Stirm, Lina Marie Fonteyne, Bachuki Shashikadze, Magdalena Lindner, Maila Chirivi, Andreas Lange, Clara Kaufhold, Christian Mayer, Ivica Medugorac, Barbara Kessler and 25 more

Open access · goldAbstract read
In one paragraph

Article in Disease models & mechanisms, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers.

0numbers the graph read from it
0cells of the map it votes in
35citing papers in PubMed
2.4field-weighted citation impact, top 10% 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

35 citing papers in PubMed, 44 citations in OpenAlex.

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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

35 authors at 10 institutions in 3 countries.

Michael StirmChair for Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, 81377 Munich, Germany.
Lina Marie FonteyneChair for Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, 81377 Munich, Germany.
Bachuki ShashikadzeLaboratory for Functional Genome Analysis (LAFUGA), Gene Center, LMU Munich, 81377 Munich, Germany.
Magdalena LindnerDepartment of Nuclear Medicine, University Hospital, LMU Munich, 81377 Munich, Germany.
Maila ChiriviFondazione Istituto Nazionale di Genetica Molecolare, 20122 Milan, Italy.
Andreas LangeChair for Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, 81377 Munich, Germany.
Clara KaufholdInstitute of Veterinary Pathology, Center for Clinical Veterinary Medicine, LMU Munich, 80539 Munich, Germany.
Christian MayerInstitute of Veterinary Pathology, Center for Clinical Veterinary Medicine, LMU Munich, 80539 Munich, Germany.
Ivica MedugoracPopulation Genomics Group, Department of Veterinary Sciences, LMU Munich, 82152 Martinsried, Germany.
Barbara KesslerChair for Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, 81377 Munich, Germany.
Mayuko KuromeChair for Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, 81377 Munich, Germany.
Valeri ZakhartchenkoChair for Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, 81377 Munich, Germany.
Arne HinrichsChair for Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, 81377 Munich, Germany.
Elisabeth KemterChair for Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, 81377 Munich, Germany.
Sabine KrauseFriedrich Baur Institute, Department of Neurology, LMU Munich, 80336 Munich, Germany.
Rüdiger WankeInstitute of Veterinary Pathology, Center for Clinical Veterinary Medicine, LMU Munich, 80539 Munich, Germany.
Georg J ArnoldLaboratory for Functional Genome Analysis (LAFUGA), Gene Center, LMU Munich, 81377 Munich, Germany.
Gerhard WessClinic of Small Animal Medicine, Center for Clinical Veterinary Medicine, LMU Munich, 80539 Munich, Germany.
Hiroshi NagashimaMeiji University International Institute for Bio-Resource Research, 214-8571 Kawasaki, Japan.
Martin Hrabĕ de AngelisInstitute of Experimental Genetics, Helmholtz Zentrum Munich, 85764 Neuherberg, Germany.
Florian FlenkenthalerLaboratory for Functional Genome Analysis (LAFUGA), Gene Center, LMU Munich, 81377 Munich, Germany.
Levin Arne KobelkeLaboratory for Functional Genome Analysis (LAFUGA), Gene Center, LMU Munich, 81377 Munich, Germany.
Claudia BearziFondazione Istituto Nazionale di Genetica Molecolare, 20122 Milan, Italy.
Roberto RizziFondazione Istituto Nazionale di Genetica Molecolare, 20122 Milan, Italy.
Andrea BährKlinik und Poliklinik für Innere Medizin I, Klinikum rechts der Isar, Technical University Munich and German Center for Cardiovascular Research (DZHK), Munich Heart Alliance, 81675 Munich, Germany.
Sven ReeseChair for Anatomy, Histology and Embryology, Department of Veterinary Sciences, LMU Munich, 80539 Munich, Germany.
Kaspar MatiasekInstitute of Veterinary Pathology, Center for Clinical Veterinary Medicine, LMU Munich, 80539 Munich, Germany.
Maggie C WalterFriedrich Baur Institute, Department of Neurology, LMU Munich, 80336 Munich, Germany.
Christian KupattKlinik und Poliklinik für Innere Medizin I, Klinikum rechts der Isar, Technical University Munich and German Center for Cardiovascular Research (DZHK), Munich Heart Alliance, 81675 Munich, Germany.
Sibylle ZieglerDepartment of Nuclear Medicine, University Hospital, LMU Munich, 81377 Munich, Germany.
Peter BartensteinDepartment of Nuclear Medicine, University Hospital, LMU Munich, 81377 Munich, Germany.
Thomas FröhlichLaboratory for Functional Genome Analysis (LAFUGA), Gene Center, LMU Munich, 81377 Munich, Germany.
Nikolai KlymiukChair for Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, 81377 Munich, Germany.
Andreas BlutkeInstitute of Experimental Genetics, Helmholtz Zentrum Munich, 85764 Neuherberg, Germany.
Eckhard WolfChair for Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, 81377 Munich, Germany.ORCID 0000-0002-0430-9510
Ludwig-Maximilians-Universität München · DEInstitute of Bioinformatics and Systems Biology · DELMU Klinikum · DEFriedrich Baur Stiftung · DEHelmholtz Zentrum München · DETUM Klinikum · DEInstitute of Biomedical Technologies · ITInstitute of Genetic and Biomedical Research · ITIstituto Nazionale Genetica Molecolare · ITMeiji University · JP

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Large-animal models for Duchenne muscular dystrophy (DMD) are crucial for the evaluation of diagnostic procedures and treatment strategies. Pigs cloned from male cells lacking DMD exon 52 (DMDΔ52) exhibit molecular, clinical and pathological hallmarks of DMD, but die before sexual maturity and cannot be propagated by breeding. Therefore, we generated female DMD+/- carriers. A single founder animal had 11 litters with 29 DMDY/-, 34 DMD+/- as well as 36 male and 29 female wild-type offspring. Breeding with F1 and F2 DMD+/- carriers resulted in an additional 114 DMDY/- piglets. With intensive neonatal management, the majority survived for 3-4 months, providing statistically relevant cohorts for experimental studies. Pathological investigations and proteome studies of skeletal muscles and myocardium confirmed the resemblance to human disease mechanisms. Importantly, DMDY/- pigs displayed progressive myocardial fibrosis and increased expression of connexin-43, associated with significantly reduced left ventricular ejection fraction, at 3 months. Furthermore, behavioral tests provided evidence for impaired cognitive ability. Our breeding cohort of DMDΔ52 pigs and standardized tissue repositories provide important resources for studying DMD disease mechanisms and for testing novel treatment strategies.

Indexed as

CardiomyopathiesMuscular Dystrophy, DuchenneAnimalsFemaleHumansMaleMuscle, SkeletalStroke VolumeSwineVentricular Function, LeftBiobankCarrierDuchenne muscular dystrophyPathologyPig modelProteomics

Identifiers

PMID34796900
PMCPMC8688409
OpenAlexW3213026873

What OpenQuestion holds

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LicenceCC BY
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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.