ArticleProceedings of the National Academy of Sciences of the United States of America2012
Zebrafish model for congenital myasthenic syndrome reveals mechanisms causal to developmental recovery.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 18 citations in OpenAlex.
- Article
- Allosteric inhibition of muscle-type nicotinic acetylcholine receptors by a neuromuscular blocking agent pancuronium.PloS one · 2023Article
- Modeling neuromuscular diseases in zebrafish.Frontiers in molecular neuroscience · 2022Review
- Receptor subunit compositions underly distinct potencies of a muscle relaxant in fast and slow muscle fibers.Frontiers in physiology · 2022Article
- Synaptic silencing of fast muscle is compensated by rewired innervation of slow muscle.Science advances · 2020Article
- Zebrafish neuromuscular junction: The power of N.Neuroscience letters · 2019Review
- Sequence Variations in pxr (nr1i2) From Zebrafish (Danio rerio) Strains Affect Nuclear Receptor Function.Toxicological sciences : an official journal of the Society of Toxicology · 2019Article
- Fatigue in Rapsyn-Deficient Zebrafish Reflects Defective Transmitter Release.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2016Article
- Zebrafish mutants of the neuromuscular junction: swimming in the gene pool.The journal of physiological sciences : JPS · 2015Review
- A single mutation in the acetylcholine receptor δ-subunit causes distinct effects in two types of neuromuscular synapses.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2014Article
- Neuromuscular disorders in zebrafish: state of the art and future perspectives.Neuromolecular medicine · 2013Review
Corrections and comments
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Authors and funding
8 authors at 3 institutions in 1 country.
Funding
Abstract
Mutations in muscle ACh receptors cause slow-channel syndrome (SCS) and Escobar syndrome, two forms of congenital myasthenia. SCS is a dominant disorder with mutations reported for all receptor subunits except γ. Escobar syndrome is distinct, with mutations located exclusively in γ, and characterized by developmental improvement of muscle function. The zebrafish mutant line, twister, models SCS in terms of a dominant mutation in the α subunit (α(twi)) but shows the behavioral improvement associated with Escobar syndrome. Here, we present a unique electrophysiological study into developmental improvement for a myasthenic syndrome. The embryonic α(twi)βδγ receptor isoform produces slowly decaying synaptic currents typical of SCS that transit to a much faster decay upon the appearance of adult ε, despite the α(twi) mutation. Thus, the continued expression of α(twi) into adulthood is tolerated because of the ε expression and associated recovery, raising the likelihood of unappreciated myasthenic cases that benefit from the γ-ε switch.
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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.