ArticleCommunications biology2020
Upregulation of large myelin protein zero leads to Charcot-Marie-Tooth disease-like neuropathy in mice.
Article in Communications biology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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14 citing papers in PubMed, 38 citations in OpenAlex.
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- Age-related transcriptional drift and physiological adaptation in long-living Ames dwarf skeletal muscle.NAR molecular medicine · 2026Article
- The role of secretome from mesenchymal stromal cells in promoting nerve regeneration after neurotmesis.Stem cell research & therapy · 2026Article
- Novel Dominant Splicing Variant in MPZ Associated With Unusual Charcot-Marie-Tooth Disease.Journal of the peripheral nervous system : JPNS · 2025Article
- [The damaging effects of two exposures to 100 dB white noise on myelination of the auditory nerve in mouse cochleae mechanism of repeated noise exposure on myelin sheath of cochlear auditory nerve in mice].Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery · 2025Article
- Prevention of Chronic Diabetic Neuropathy and Diabetes-Associated Cognitive Impairment Using Medicinal Herbs (The Yale journal of biology and medicine · 2024Article
- Transcriptome Profiling of miRNA-mRNA Interactions and Associated Mechanisms in Chemotherapy-Induced Neuropathic Pain.Molecular neurobiology · 2023Article
- Blocking insulin-like growth factor 1 receptor signaling pathway inhibits neuromuscular junction regeneration after botulinum toxin-A treatment.Cell death & disease · 2023Article
- Downregulation of PMP22 ameliorates myelin defects in iPSC-derived human organoid cultures of CMT1A.Brain : a journal of neurology · 2023Article
- MPZ gene variant site in Chinese patients with Charcot-Marie-Tooth disease.Molecular genetics & genomic medicine · 2022Article
- Stop codon readthrough alters the activity of a POU/Oct transcription factor during Drosophila development.BMC biology · 2021Article
- Macroscopic detection of demyelinated lesions in mouse PNS with neutral red dye.Scientific reports · 2021Article
- Stop codon read-through of mammalianThe Journal of biological chemistry · 2020Article
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Charcot-Marie-Tooth (CMT) disease is a hereditary neuropathy mainly caused by gene mutation of peripheral myelin proteins including myelin protein zero (P0, MPZ). Large myelin protein zero (L-MPZ) is an isoform of P0 that contains an extended polypeptide synthesized by translational readthrough at the C-terminus in tetrapods, including humans. The physiological role of L-MPZ and consequences of an altered L-MPZ/P0 ratio in peripheral myelin are not known. To clarify this, we used genome editing to generate a mouse line (L-MPZ mice) that produced L-MPZ instead of P0. Motor tests and electrophysiological, immunohistological, and electron microscopy analyses show that homozygous L-MPZ mice exhibit CMT-like phenotypes including thin and/or loose myelin, increased small-caliber axons, and disorganized axo-glial interactions. Heterozygous mice show a milder phenotype. These results highlight the importance of an appropriate L-MPZ/P0 ratio and show that aberrant readthrough of a myelin protein causes neuropathy.
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