ArticleThe Journal of neuroscience : the official journal of the Society for Neuroscience2004
BetaIV spectrins are essential for membrane stability and the molecular organization of nodes of Ranvier.
Article in The Journal of neuroscience : the official journal of the Society for Neuroscience, 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 88 papers.
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Who cites it
88 citing papers in PubMed, 134 citations in OpenAlex.
- Age-related decline in nuclear envelope LINC complex drives neuronal aging via axon initial segment dysfunction.EMBO reports · 2026Article
- Cytoskeletal scaffolding of NaCurrent opinion in cell biology · 2025Review
- Natural history of SPTBN4-related neurodevelopmental disorder with hypotonia, neuropathy, and deafness.Orphanet journal of rare diseases · 2025Article
- Postsynaptic β1 spectrin maintains NaThe Journal of physiology · 2024Article
- Identifying the Phenotypes of Diffuse Axonal Injury Following Traumatic Brain Injury.Brain sciences · 2023Review
- Expression patterns of NKCC1 in neurons and non-neuronal cells during cortico-hippocampal development.Cerebral cortex (New York, N.Y. : 1991) · 2023Article
- Spectrins: molecular organizers and targets of neurological disorders.Nature reviews. Neuroscience · 2023Review
- Extracellular Tau Oligomers Damage the Axon Initial Segment.Journal of Alzheimer's disease : JAD · 2023Article
- Concussion leads to widespread axonal sodium channel loss and disruption of the node of Ranvier.Acta neuropathologica · 2022Article
- Loss of β4-spectrin impairs NaScientific reports · 2022Article
- Regulation of Cardiac Conduction and Arrhythmias by Ankyrin/Spectrin-Based Macromolecular Complexes.Journal of cardiovascular development and disease · 2021Review
- Age-dependent ataxia and neurodegeneration caused by an αII spectrin mutation with impaired regulation of its calpain sensitivity.Scientific reports · 2021Article
- Mechanisms of node of Ranvier assembly.Nature reviews. Neuroscience · 2021Review
- Nodes of Ranvier during development and repair in the CNS.Nature reviews. Neurology · 2020Review
- Article
- Nodal β spectrins are required to maintain NaeLife · 2020Article
- Axonal Spectrins: Nanoscale Organization, Functional Domains and Spectrinopathies.Frontiers in cellular neuroscience · 2019Review
- An axon initial segment is required for temporal precision in action potential encoding by neuronal populations.Science advances · 2018Article
- βIV Spectrinopathies Cause Profound Intellectual Disability, Congenital Hypotonia, and Motor Axonal Neuropathy.American journal of human genetics · 2018Article
- Axon initial segments: structure, function, and disease.Annals of the New York Academy of Sciences · 2018Review
28 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
5 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
High densities of sodium channels at nodes of Ranvier permit action potential conduction and depend on betaIV spectrins, a family of scaffolding proteins linked to the cortical actin cytoskeleton. To investigate the molecular organization of nodes, we analyzed qv(3J)"quivering" mice, whose betaIV spectrins have a truncated proline-rich "specific" domain (SD) and lack the pleckstrin homology (PH) domain. Central nodes of qv(3J) mice, which lack betaIV spectrins, are significantly broader and have prominent vesicle-filled nodal membrane protrusions, whereas axon shape and neurofilament density are dramatically altered. PNS qv(3J) nodes, some with detectable betaIV spectrins, are less affected. In contrast, a larger truncation of betaIV spectrins in qv(4J) mice, deleting the SD, PH, and ankyrinG binding domains, causes betaIV spectrins to be undetectable and causes dramatic changes, even in peripheral nodes. These results show that quivering mutations disrupt betaIV spectrin retention and stability at nodes and that distinct protein domains regulate nodal structural integrity and molecular organization.
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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.