ArticleHuman molecular genetics2011
Impaired muscle growth and response to insulin-like growth factor 1 in dysferlin-mediated muscular dystrophy.
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 44 papers.
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Who cites it
44 citing papers in PubMed, 70 citations in OpenAlex.
- DAB2 in LGMD R2: a molecular link between disease progression and lipid dysregulation.JCI insight · 2026Article
- Metabolic dysregulation contributes to the development of dysferlinopathy.Life science alliance · 2025Article
- Syntaxin 4-enhanced plasma membrane repair is independent of dysferlin in skeletal muscle.American journal of physiology. Cell physiology · 2025Article
- Biasing the conformation of ELMO2 reveals that myoblast fusion can be exploited to improve muscle regeneration.Nature communications · 2022Article
- Loss of dysferlin or myoferlin results in differential defects in excitation-contraction coupling in mouse skeletal muscle.Scientific reports · 2021Article
- The Dysferlin Transcript Containing the Alternative Exon 40a is Essential for Myocyte Functions.Frontiers in cell and developmental biology · 2021Article
- Functions of Vertebrate Ferlins.Cells · 2020Review
- Treatment with galectin-1 improves myogenic potential and membrane repair in dysferlin-deficient models.PloS one · 2020Article
- Pulsed glucocorticoids enhance dystrophic muscle performance through epigenetic-metabolic reprogramming.JCI insight · 2019Observational
- Defects in G-Actin Incorporation into Filaments in Myoblasts Derived from Dysferlinopathy Patients Are Restored by Dysferlin C2 Domains.International journal of molecular sciences · 2019Article
- Engineered skeletal muscles for disease modeling and drug discovery.Biomaterials · 2019Review
- Functional muscle hypertrophy by increased insulin-like growth factor 1 does not require dysferlin.Muscle & nerve · 2019Article
- Emerging Functional Differences between the Synaptotagmin and Ferlin Calcium Sensor Families.Biochemistry · 2017Article
- Intermittent Glucocorticoid Dosing Improves Muscle Repair and Function in Mice with Limb-Girdle Muscular Dystrophy.The American journal of pathology · 2017Article
- Treatment with Recombinant Human MG53 Protein Increases Membrane Integrity in a Mouse Model of Limb Girdle Muscular Dystrophy 2B.Molecular therapy : the journal of the American Society of Gene Therapy · 2017Article
- Muscle cell communication in development and repair.Current opinion in pharmacology · 2017Review
- Co-expression of truncated and full-length tau induces severe neurotoxicity.Molecular psychiatry · 2016Article
- An actin-dependent annexin complex mediates plasma membrane repair in muscle.The Journal of cell biology · 2016Article
- Enhanced Muscular Dystrophy from Loss of Dysferlin Is Accompanied by Impaired Annexin A6 Translocation after Sarcolemmal Disruption.The American journal of pathology · 2016Article
- Plasma Membrane Repair in Health and Disease.Current topics in membranes · 2016Review
Corrections and comments
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
Abstract
Loss-of-function mutations in dysferlin cause muscular dystrophy, and dysferlin has been implicated in resealing membrane disruption in myofibers. Given the importance of membrane fusion in many aspects of muscle function, we studied the role of dysferlin in muscle growth. We found that dysferlin null myoblasts have a defect in myoblast-myotube fusion, resulting in smaller myotubes in culture. In vivo, dysferlin null muscle was found to have mislocalized nuclei and vacuolation. We found that myoblasts isolated from dysferlin null mice accumulate enlarged, lysosomal-associated membrane protein 2 (LAMP2)-positive lysosomes. Dysferlin null myoblasts accumulate transferrin-488, reflecting abnormal vesicular trafficking. Additionally, dysferlin null myoblasts display abnormal trafficking of the insulin-like growth factor (IGF) receptor, where the receptor is shuttled to LAMP2-positive lysosomes. We studied growth, in vivo, by infusing mice with the growth stimulant IGF1. Control IGF1-treated mice increased myofiber diameter by 30% as expected, whereas dysferlin null muscles had no response to IGF1, indicating a defect in myofiber growth. We also noted that dysferlin null fibroblasts also accumulate acidic vesicles, IGF receptor and transferrin, indicating that dysferlin is important for nonmuscle vesicular trafficking. These data implicate dysferlin in multiple membrane fusion events within the cell and suggest multiple pathways by which loss of dysferlin contributes to muscle disease.
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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.