ArticleDisease models & mechanisms2012
Neb: a zebrafish model of nemaline myopathy due to nebulin mutation.
Article in Disease models & mechanisms, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers.
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Who cites it
37 citing papers in PubMed, 48 citations in OpenAlex.
- Identifying kinematic biomarkers of the dystrophic phenotype in a zebrafish model of Duchenne muscular dystrophy.Skeletal muscle · 2025Article
- High resolution kinematic approach for quantifying impaired mobility of dystrophic zebrafish larvae.bioRxiv : the preprint server for biology · 2024Article
- Aligning with the 3Rs: alternative models for research into muscle development and inherited myopathies.BMC veterinary research · 2024Review
- Comprehensive phenotypic characterization of an allelic series of zebrafish models of NEB-related nemaline myopathy.Human molecular genetics · 2024Article
- Characterization of NEB pathogenic variants in patients reveals novel nemaline myopathy disease mechanisms and omecamtiv mecarbil force effects.Acta neuropathologica · 2024Article
- NRAP reduction rescues sarcomere defects in nebulin-related nemaline myopathy.Human molecular genetics · 2023Article
- Modeling Human Muscular Dystrophies in Zebrafish: Mutant Lines, Transgenic Fluorescent Biosensors, and Phenotyping Assays.International journal of molecular sciences · 2023Review
- A forward genetic screen identifies Dolk as a regulator of startle magnitude through the potassium channel subunit Kv1.1.PLoS genetics · 2021Article
- Zebrafish models of sarcopenia.Disease models & mechanisms · 2020Review
- Nebulin: big protein with big responsibilities.Journal of muscle research and cell motility · 2020Review
- Zebrafish Models ofFrontiers in molecular neuroscience · 2020Article
- Is Gene-Size an Issue for the Diagnosis of Skeletal Muscle Disorders?Journal of neuromuscular diseases · 2020Review
- Zebrafish structural development in Mueller-matrix scanning microscopy.Scientific reports · 2019Article
- Failure to identify modifiers ofBiology open · 2019Article
- Testing of therapies in a novel nebulin nemaline myopathy model demonstrate a lack of efficacy.Acta neuropathologica communications · 2018Article
- NO-sGC Pathway Modulates CaFrontiers in physiology · 2017Article
- Using Touch-evoked Response and Locomotion Assays to Assess Muscle Performance and Function in Zebrafish.Journal of visualized experiments : JoVE · 2016Article
- Analysis of Zebrafish Larvae Skeletal Muscle Integrity with Evans Blue Dye.Journal of visualized experiments : JoVE · 2015Article
- In vivo assessment of contractile strength distinguishes differential gene function in skeletal muscle of zebrafish larvae.Journal of applied physiology (Bethesda, Md. : 1985) · 2015Article
- Zebrafish models for nemaline myopathy reveal a spectrum of nemaline bodies contributing to reduced muscle function.Acta neuropathologica · 2015Article
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
Abstract
Nemaline myopathy is one of the most common and severe non-dystrophic muscle diseases of childhood. Patients typically present in infancy with hypotonia, weakness, delayed motor development, and bulbar and respiratory difficulties. Mutations in six different genes are associated with nemaline myopathy, with nebulin mutations being the most common. No treatments or disease-modifying therapies have been identified for this disease. One of the major barriers to treatment development is the lack of models amenable to rapid and coordinated testing of potential therapeutic strategies. To overcome this barrier, we have characterized the first zebrafish model of nemaline myopathy. This model, termed neb, harbors a recessive mutation in the nebulin gene that results in decreased Nebulin protein levels, a severe motor phenotype and premature lethality. In addition to impaired motor function, neb zebrafish exhibit many of the features associated with human nemaline myopathy. These include impaired force generation, altered thin filament length and the presence of specific histopathological changes, including the formation of nemaline bodies. In summary, neb zebrafish mirror the genetic, clinical and pathological aspects of nemaline myopathy due to NEB mutation, and thus are an excellent model for future therapy development for this devastating disorder.
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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.