ArticleThe Journal of cell biology2000
Epitope-tagged P(0) glycoprotein causes Charcot-Marie-Tooth-like neuropathy in transgenic mice.
Article in The Journal of cell biology, 2000. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 57 citations in OpenAlex.
- Homomeric interactions of the MPZ Ig domain and their relation to Charcot-Marie-Tooth disease.Brain : a journal of neurology · 2023Article
- A novel mouse model of CMT1B identifies hyperglycosylation as a new pathogenetic mechanism.Human molecular genetics · 2022Article
- Animal Models as a Tool to Design Therapeutical Strategies for CMT-like Hereditary Neuropathies.Brain sciences · 2021Review
- Imaging MyelinationBrain plasticity (Amsterdam, Netherlands) · 2016Review
- Transgene expression in the Nop-tTA driver line is not inherently restricted to the entorhinal cortex.Brain structure & function · 2016Article
- Virus-mediated swapping of zolpidem-insensitive with zolpidem-sensitive GABA(A) receptors in cortical pyramidal cells.The Journal of physiology · 2012Article
- Article
- P0S63del impedes the arrival of wild-type P0 glycoprotein to myelin in CMT1B mice.Human molecular genetics · 2011Article
- Article
- Induction of myelin protein zero by early growth response 2 through upstream and intragenic elements.The Journal of biological chemistry · 2009Article
- Haploinsufficiency of AFG3L2, the gene responsible for spinocerebellar ataxia type 28, causes mitochondria-mediated Purkinje cell dark degeneration.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2009Article
- Peripheral myelin of Xenopus laevis: role of electrostatic and hydrophobic interactions in membrane compaction.Journal of structural biology · 2008Article
- The mitochondrial protease AFG3L2 is essential for axonal development.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2008Article
- Ablation of the UPR-mediator CHOP restores motor function and reduces demyelination in Charcot-Marie-Tooth 1B mice.Neuron · 2008Article
- Production of endothelial nitric oxide synthase (eNOS) over-expressing piglets.Transgenic research · 2006Article
- Different intracellular pathomechanisms produce diverse Myelin Protein Zero neuropathies in transgenic mice.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2006Article
- Intramuscular viral delivery of paraplegin rescues peripheral axonopathy in a model of hereditary spastic paraplegia.The Journal of clinical investigation · 2006Article
- Pathomechanisms of mutant proteins in Charcot-Marie-Tooth disease.Neuromolecular medicine · 2006Review
- Loss of Mtmr2 phosphatase in Schwann cells but not in motor neurons causes Charcot-Marie-Tooth type 4B1 neuropathy with myelin outfoldings.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2005Article
- Axonal degeneration in paraplegin-deficient mice is associated with abnormal mitochondria and impairment of axonal transport.The Journal of clinical investigation · 2004Article
Corrections and comments
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Authors and funding
11 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
In peripheral nerve myelin, the intraperiod line results from compaction of the extracellular space due to homophilic adhesion between extracellular domains (ECD) of the protein zero (P(0)) glycoprotein. Point mutations in this region of P(0) cause human hereditary demyelinating neuropathies such as Charcot-Marie-Tooth. We describe transgenic mice expressing a full-length P(0) modified in the ECD with a myc epitope tag. The presence of the myc sequence caused a dysmyelinating peripheral neuropathy similar to two distinct subtypes of Charcot-Marie-Tooth, with hypomyelination, altered intraperiod lines, and tomacula (thickened myelin). The tagged protein was incorporated into myelin and was associated with the morphological abnormalities. In vivo and in vitro experiments showed that P(0)myc retained partial adhesive function, and suggested that the transgene inhibits P(0)-mediated adhesion in a dominant-negative fashion. These mice suggest new mechanisms underlying both the pathogenesis of P(0) ECD mutants and the normal interactions of P(0) in the myelin sheath.
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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.