ArticleHuman molecular genetics2011
Muscle-specific expression of insulin-like growth factor 1 improves outcome in Lama2Dy-w mice, a model for congenital muscular dystrophy type 1A.
Article in Human molecular genetics, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 42 citations in OpenAlex.
- Emerging roles of microRNAs and other non-coding transcriptome in muscular dystrophies.Inflammation and regeneration · 2026Review
- Spatial proteomics reveals recombinant human laminin-111 restores adhesion signaling to laminin-α2-deficient muscle.JCI insight · 2025Article
- Characteristics of bovine muscle satellite cell from different breeds for efficient production of cultured meat.Journal of animal science and technology · 2024Article
- Donor Hepatic Occult Collagen Deposition Predisposes to Peritransplant Stress and Impacts Human Liver Transplantation.Hepatology (Baltimore, Md.) · 2021Article
- The non-muscle ADF/cofilin-1 controls sarcomeric actin filament integrity and force production in striated muscle laminopathies.Cell reports · 2021Article
- Micro-laminin gene therapy can function as an inhibitor of muscle disease in the dyMolecular therapy. Methods & clinical development · 2021Article
- Review
- Transcriptomic Profile of Primary Culture of Skeletal Muscle Cells Isolated from Semitendinosus Muscle of Beef and Dairy Bulls.International journal of molecular sciences · 2020Article
- A Family of Laminin α2 Chain-Deficient Mouse Mutants: Advancing the Research on LAMA2-CMD.Frontiers in molecular neuroscience · 2020Review
- Early skeletal muscle pathology and disease progress in the dyScientific reports · 2019Article
- Current understanding and treatment of cardiac and skeletal muscle pathology in laminin-α2 chain-deficient congenital muscular dystrophy.The application of clinical genetics · 2019Article
- Absence of microRNA-21 does not reduce muscular dystrophy in mouse models of LAMA2-CMD.PloS one · 2017Article
- miR-410 and miR-495 Are Dynamically Regulated in Diverse Cardiomyopathies and Their Inhibition Attenuates Pathological Hypertrophy.PloS one · 2016Article
- Do's and don'ts in the preparation of muscle cryosections for histological analysis.Journal of visualized experiments : JoVE · 2015Article
- Article
- Combining immunodetection with histochemical techniques: the effect of heat-induced antigen retrieval on picro-Sirius red staining.The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society · 2014Article
- Quantitative proteomic analysis reveals metabolic alterations, calcium dysregulation, and increased expression of extracellular matrix proteins in laminin α2 chain-deficient muscle.Molecular & cellular proteomics : MCP · 2014Article
- Systemic administration of a recombinant AAV1 vector encoding IGF-1 improves disease manifestations in SMA mice.Molecular therapy : the journal of the American Society of Gene Therapy · 2014Article
- Laminin-111 improves muscle repair in a mouse model of merosin-deficient congenital muscular dystrophy.Human molecular genetics · 2014Article
- Dysregulation of matricellular proteins is an early signature of pathology in laminin-deficient muscular dystrophy.Skeletal muscle · 2014Article
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
MDC1A, the second most prevalent form of congenital muscular dystrophy, results from laminin-α2 chain deficiency. This disease is characterized by extensive muscle wasting that results in extremely weak skeletal muscles. A large percentage of children with MDC1A are faced with respiratory as well as ambulatory difficulties. We investigated the effects of overexpressing insulin-like growth factor-1 (IGF-1) as a potential therapeutic target for the disease in the Lama2(Dy-w) mouse, a model that closely resembles human MDC1A. IGF-1 transgenic Lama2(Dy-w) mice showed increased survivability, body weight and muscle weight. In addition, these mice showed better ability to stand up on their hind limbs: a typical exploratory behavior seen in healthy mice. Histology and immunohistochemistry analyses revealed increased regenerative capacity and proliferation in IGF-1 transgenic Lama2(Dy-w) muscles. Western blot analysis showed increased phosphorylation of Akt and ERK1/2, both known to enhance myogenesis. Additionally, we saw increases in the expression of the regeneration markers MyoD, myogenin and embryonic myosin (myosin heavy chain 3, MYH3). We conclude that overexpression of IGF-1 in Lama2(Dy-w) mice increases lifespan and improves their overall wellbeing mainly through the restoration of impaired muscle regeneration, as fibrosis or inflammation was not impacted by IGF-1 in this disease model. Our results demonstrate that IGF-1 has a promising therapeutic potential in the treatment of MDC1A.
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