ArticleGlia2008
Remodeling of motor nerve terminals in demyelinating axons of periaxin-null mice.
Article in Glia, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 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
18 citing papers in PubMed, 32 citations in OpenAlex.
- Junctions in Jeopardy: the neuromuscular junction is a selective pathological target in Charcot-Marie-Tooth disease.Mammalian genome : official journal of the International Mammalian Genome Society · 2026Review
- Boosting BDNF in muscle rescues impaired axonal transport in a mouse model of DI-CMTC peripheral neuropathy.Neurobiology of disease · 2024Article
- Boosting BDNF in muscle rescues impaired axonal transport in a mouse model of DI-CMTC peripheral neuropathy.bioRxiv : the preprint server for biology · 2024Article
- A SARM1-mitochondrial feedback loop drives neuropathogenesis in a Charcot-Marie-Tooth disease type 2A rat model.The Journal of clinical investigation · 2022Article
- Confocal Endomicroscopy of Neuromuscular Junctions Stained with Physiologically Inert Protein Fragments of Tetanus Toxin.Biomolecules · 2021Article
- Developmental demands contribute to early neuromuscular degeneration in CMT2D mice.Cell death & disease · 2020Article
- Morphological remodeling during recovery of the neuromuscular junction from terminal Schwann cell ablation in adult mice.Scientific reports · 2020Article
- Impaired Mitophagy Plays a Role in Denervation of Neuromuscular Junctions in ALS Mice.Frontiers in neuroscience · 2017Article
- Intermolecular disulfide bond in the dimerization of S-periaxin mediated by Cys88 and Cys139.Acta biochimica et biophysica Sinica · 2016Article
- Loss of glial neurofascin155 delays developmental synapse elimination at the neuromuscular junction.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2014Article
- Altered active zones, vesicle pools, nerve terminal conductivity, and morphology during experimental MuSK myasthenia gravis.PloS one · 2014Article
- Charcot-Marie-Tooth disease and intracellular traffic.Progress in neurobiology · 2012Review
- Curcumin derivatives promote Schwann cell differentiation and improve neuropathy in R98C CMT1B mice.Brain : a journal of neurology · 2012Article
- A mouse model of Schwartz-Jampel syndrome reveals myelinating Schwann cell dysfunction with persistent axonal depolarization in vitro and distal peripheral nerve hyperexcitability when perlecan is lacking.The American journal of pathology · 2012Article
- Ventilatory impairment in the dysmyelinated Long Evans shaker rat.Neuroscience · 2010Article
- The contributions of myelin and axonal caliber to transverse relaxation time in shiverer and neurofilament-deficient mouse models.NeuroImage · 2010Article
- Congenital hypomyelinating neuropathy with lethal conduction failure in mice carrying the Egr2 I268N mutation.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2009Article
- Ablation of the UPR-mediator CHOP restores motor function and reduces demyelination in Charcot-Marie-Tooth 1B mice.Neuron · 2008Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
Myelin formation around axons increases nerve conduction velocity and influences both the structure and function of the myelinated axon. In the peripheral nervous system, demyelinating forms of hereditary Charcot-Marie-Tooth (CMT) diseases cause reduced nerve conduction velocity initially and ultimately axonal degeneration. Several mouse models of CMT diseases have been generated, allowing the study of the consequences of disrupting Schwann cell function on peripheral nerve fibers. Nevertheless, the effect of demyelination at the level of the neuromuscular synapse has been largely overlooked. Here we show that in mice lacking functional Periaxin (Prx) genes, a model of a recessive type of CMT disease known as CMT4F, neuromuscular junctions (NMJs) develop profound morphological changes in the preterminal region of motor axons. These changes include extensive preterminal branches that originate in demyelinated regions of the nerve fiber and axonal swellings associated with residually-myelinated regions of the fiber. Using intracellular recording from muscle fibers we detected asynchronous failure of action potential transmission at high but not low stimulation frequencies, a phenomenon consistent with branch point failure. Taken together, our morphological and electrophysiological findings suggest that preterminal branching due to segmental demyelination near the neuromuscular synapse in Periaxin KO mice may underlie some characteristics of disabilities, including coordination deficits, present in this mouse model of CMT disease. These results reveal the importance of studying how demyelinating diseases might influence NMJ function and contribute to clinical disability.
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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.