ArticlePLoS currents2012
Dysferlin-deficient immortalized human myoblasts and myotubes as a useful tool to study dysferlinopathy.
Article in PLoS currents, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
What it found
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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.
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.
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Who cites it
22 citing papers in PubMed, 37 citations in OpenAlex.
- DAB2 in LGMD R2: a molecular link between disease progression and lipid dysregulation.JCI insight · 2026Article
- Engineering Functional Skeletal Muscle Tissues from Human Induced Pluripotent Stem Cells.Methods in molecular biology (Clifton, N.J.) · 2026Article
- Impaired myogenesis in limb girdle muscular dystrophy type 2B.Scientific reports · 2025Article
- Antisense oligonucleotide-mediated exon 27 skipping restores dysferlin function in dysferlinopathy patient-derived muscle cells.Molecular therapy. Nucleic acids · 2025Article
- A Novel Assay Reveals the Early Setting-Up of Membrane Repair Machinery in Human Skeletal Muscle Cells.Journal of cellular biochemistry · 2025Article
- Bioengineered Model of Human LGMD2B Skeletal Muscle Reveals Roles of Intracellular Calcium Overload in Contractile and Metabolic Dysfunction in Dysferlinopathy.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2024Article
- The Dysferlinopathies Conundrum: Clinical Spectra, Disease Mechanism and Genetic Approaches for Treatments.Biomolecules · 2024Review
- Skeletal Muscle Cells Derived from Induced Pluripotent Stem Cells: A Platform for Limb Girdle Muscular Dystrophies.Biomedicines · 2022Article
- Therapeutic Benefit of Galectin-1: Beyond Membrane Repair, a Multifaceted Approach to LGMD2B.Cells · 2021Article
- AMPK Complex Activation Promotes Sarcolemmal Repair in Dysferlinopathy.Molecular therapy : the journal of the American Society of Gene Therapy · 2020Article
- Functions of Vertebrate Ferlins.Cells · 2020Review
- 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
- Cell Membrane Repair Assay Using a Two-photon Laser Microscope.Journal of visualized experiments : JoVE · 2018Article
- Serum exosomes can restore cellular functionTheranostics · 2018Article
- IRS4, a novel modulator of BMP/Smad and Akt signalling during early muscle differentiation.Scientific reports · 2017Article
- Skeletal muscle characteristics are preserved in hTERT/cdk4 human myogenic cell lines.Skeletal muscle · 2016Article
- The absence of dysferlin induces the expression of functional connexin-based hemichannels in human myotubes.BMC cell biology · 2016Article
- Dysferlinopathy Fibroblasts Are Defective in Plasma Membrane Repair.PLoS currents · 2015Article
- Small molecules dorsomorphin and LDN-193189 inhibit myostatin/GDF8 signaling and promote functional myoblast differentiation.The Journal of biological chemistry · 2015Article
- Dysferlin regulates cell membrane repair by facilitating injury-triggered acid sphingomyelinase secretion.Cell death & disease · 2014Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
6 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Dysferlin gene mutations causing LGMD2B are associated with defects in muscle membrane repair. Four stable cell lines have been established from primary human dysferlin-deficient myoblasts harbouring different mutations in the dysferlin gene. We have compared immortalized human myoblasts and myotubes carrying disease-causing mutations in dysferlin to their wild-type counterparts. Fusion of myoblasts into myotubes and expression of muscle-specific differentiation markers were investigated with special emphasis on dysferlin protein expression, subcellular localization and function in membrane repair. We found that the immortalized myoblasts and myotubes were virtually indistinguishable from their parental cell line for all of the criteria we investigated. They therefore will provide a very useful tool to further investigate dysferlin function and pathophysiology as well as to test therapeutic strategies at the cellular level.
Identifiers
What OpenQuestion holds
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.