ArticleNeurobiology of disease2007
Axonal and periaxonal swelling precede peripheral neurodegeneration in KCC3 knockout mice.
Article in Neurobiology of disease, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.
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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.
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Who cites it
40 citing papers in PubMed, 80 citations in OpenAlex.
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- Neuron-specific ablation of the Krabbe disease gene galactosylceramidase in mice results in neurodegeneration.PLoS biology · 2022Article
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- Temporal manipulation of KCC3 expression in juvenile or adult mice suggests irreversible developmental deficit in hereditary motor sensory neuropathy with agenesis of the corpus callosum.American journal of physiology. Cell physiology · 2021Article
- Advances in the development of novel compounds targeting cation-chloride cotransporter physiology.American journal of physiology. Cell physiology · 2021Review
- Bidirectional regulation of glial potassium buffering - glioprotection versus neuroprotection.eLife · 2021Article
- Targeting the WNK-SPAK/OSR1 Pathway and Cation-Chloride Cotransporters for the Therapy of Stroke.International journal of molecular sciences · 2021Review
- Osmotic Response of Dorsal Root Ganglion Neurons Expressing Wild-Type and Mutant KCC3 Transporters.Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology · 2020Article
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- SIK3 suppresses neuronal hyperexcitability by regulating the glial capacity to buffer KThe Journal of cell biology · 2019Article
- The WNK-SPAK/OSR1 Kinases and the Cation-Chloride Cotransporters as Therapeutic Targets for Neurological Diseases.Aging and disease · 2019Review
- Physiological roles and molecular mechanisms of KThe Journal of physiology · 2019Review
- A role for KCC3 in maintaining cell volume of peripheral nerve fibers.Neurochemistry international · 2019Review
- Cell Volume Control in Healthy Brain and Neuropathologies.Current topics in membranes · 2018Review
- Water Homeostasis and Cell Volume Maintenance and Regulation.Current topics in membranes · 2018Review
- Peripheral motor neuropathy is associated with defective kinase regulation of the KCC3 cotransporter.Science signaling · 2016Article
- Oligodendrocytes Are Targets of HIV-1 Tat: NMDA and AMPA Receptor-Mediated Effects on Survival and Development.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2015Article
- K-Cl cotransporters, cell volume homeostasis, and neurological disease.Trends in molecular medicine · 2015Review
- Regulated phosphorylation of the K-Cl cotransporter KCC3 is a molecular switch of intracellular potassium content and cell volume homeostasis.Frontiers in cellular neuroscience · 2015Article
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
We have previously reported CNS and locomotor deficits in KCC3 knockout mice, an animal model of agenesis of the corpus callosum associated with peripheral neuropathy (ACCPN) [Howard, H.C., Mount, D.B., Rochefort, D., Byun, N., Dupre, N., Lu, J., Fan, X., Song, L., Riviere, J.B., Prevost, C., Horst, J., Simonati, A., Lemcke, B., Welch, R., England, R., Zhan, F.Q., Mercado, A., Siesser, W.B., George, A.L., Jr., McDonald, M.P., Bouchard, J.P., Mathieu, J., Delpire, E., Rouleau, G.A., 2002. The K-Cl cotransporter KCC3 is mutant in a severe peripheral neuropathy associated with agenesis of the corpus callosum. Nat. Genet. 32, 384-392]. To assess the role of KCC3 in peripheral axon and/or myelin development and maintenance, we determined its expression and performed a detailed morphometric analysis of sciatic nerves. Sciatic nerves of juvenile wild-type mice, but not of adult, express KCC3. In the knockout, Schwann cell/myelin development appears normal at P3, but axons are swollen. At P8 and into P30, some fibers accumulate fluid periaxonally. These initial swelling pathologies are followed by axon and myelin degeneration in adult nerves, leading to reduction in nerve conduction velocity. Mutant mice also exhibit decreased sensitivity to noxious pain. This evidence for fluid-related axonopathy, which ultimately result in neurodegeneration, implicates cell volume regulation as a critical component of peripheral nerve maintenance.
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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.