ArticleFASEB journal : official publication of the Federation of American Societies for Experimental Biology2014
Dystrophin insufficiency causes selective muscle histopathology and loss of dystrophin-glycoprotein complex assembly in pig skeletal muscle.
Article in FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.
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Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it, 29 citations in OpenAlex.
- Guideline
- Characterization of Gait Kinematics and Muscle Function in Becker Muscular Dystrophy Pigs: a pilot study.bioRxiv : the preprint server for biology · 2025Article
- The Incidence of Volatile Anesthesia Porcine Stress Syndrome in Pigs (Sus scrofa domesticus) Gives Implications for Physiology during Anesthesia.Journal of the American Association for Laboratory Animal Science : JAALAS · 2025Article
- Dystrophin (International journal of molecular sciences · 2023Article
- A Nonsense Variant in theAnimals : an open access journal from MDPI · 2022Article
- Muscular dystrophy: Experimental animal models and therapeutic approaches (Review).Experimental and therapeutic medicine · 2021Review
- Article
- Mouse models of two missense mutations in actin-binding domain 1 of dystrophin associated with Duchenne or Becker muscular dystrophy.Human molecular genetics · 2018Article
- Transcriptome analysis reveals long intergenic non-coding RNAs involved in skeletal muscle growth and development in pig.Scientific reports · 2017Article
- Oral quercetin administration transiently protects respiratory function in dystrophin-deficient mice.The Journal of physiology · 2016Article
- Current Translational Research and Murine Models For Duchenne Muscular Dystrophy.Journal of neuromuscular diseases · 2016Review
- Current Progress in Therapeutic Gene Editing for Monogenic Diseases.Molecular therapy : the journal of the American Society of Gene Therapy · 2016Review
- Animal models of Duchenne muscular dystrophy: from basic mechanisms to gene therapy.Disease models & mechanisms · 2015Review
- Porcine models of muscular dystrophy.ILAR journal · 2015Review
- Dystrophin-deficient large animal models: translational research and exon skipping.American journal of translational research · 2015Review
- Inhibition of autoimmune Chagas-like heart disease by bone marrow transplantation.PLoS neglected tropical diseases · 2014Article
- Development of rabbit monoclonal antibodies for detection of alpha-dystroglycan in normal and dystrophic tissue.PloS one · 2014Article
- Characterization of dystrophin deficient rats: a new model for Duchenne muscular dystrophy.PloS one · 2014Article
- Article
- Pharmacologic management of Duchenne muscular dystrophy: target identification and preclinical trials.ILAR journal · 2014Review
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
Abstract
The purpose of this investigation was to determine the extent to which dystrophin insufficiency caused histomorphological changes in a novel pig model of Becker muscular dystrophy. In our procedures, we used a combination of biochemical approaches, including quantitative PCR and Western blots, along with a histological analysis using standard and immunohistological measures. We found that 8-wk-old male affected pigs had a 70% reduction in dystrophin protein abundance in the diaphragm, psoas major, and longissimus lumborum and a 5-fold increase in serum creatine kinase activity compared with healthy male littermates. Dystrophin insufficiency in the diaphragm and the longissimus resulted in muscle histopathology with disorganized fibrosis that often colocalized with fatty infiltration but not the psoas. Affected animals also had an 80-85% reduction in α-sarcoglycan localization in these muscles, indicating compromised assembly of the dystrophin glycoprotein complex. Controls used in this study were 4 healthy male littermates, as they are most closely related to the affected animals. We concluded that pigs with insufficient dystrophin protein expression have a phenotype consistent with human dystrophinopathy patients. Given that and their similarity in body size and physiology to humans, we further conclude that this pig line is an appropriate translational model for dystrophinopathies.
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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.