Hereditary demyelinating neuropathy in the Trembler mouse☆
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Cited by (66)
Rodent models with expression of PMP22: Relevance to dysmyelinating CMT and HNPP
2019, Journal of the Neurological SciencesCitation Excerpt :Although a few small-caliber myelinated axons display typical myelin sheaths of a nearly normal thickness, most axons of caliber that would normally be fully myelinated have no myelin sheath or are surrounded by an abnormally thin one. There are proliferations of loose basal lamina around the SC (reminiscent of the ‘onion-bulb’ patterns) (Fig. 7A and B) [23,24]. Heterozygous mice show features of severe hypomyelination, but less pronounced than in Tr−/−.
Electrophysiological features of the mouse tail nerves and their changes in chemotherapy induced peripheral neuropathy (CIPN)
2012, Journal of Neuroscience MethodsCitation Excerpt :But they are far smaller than rats, and any neurophysiological assessment is even more difficult. Though the tail nerves should be regarded as the best chance to acquire reliable information, literature on this subject is even more sparse and heterogeneous than for rats, and is mainly focused upon motor responses (Bieri et al., 1997; Boerio et al., 2009; Low and McLeod, 1975; Rasminsky et al., 1978; Xia et al., 2010). We deemed it necessary to check that the same methods as proposed for assessment of the tail nerve conduction in the rat could be applied in the mouse with similar reliability.
Electrodiagnostic studies in a murine model of demyelinating Charcot-Marie-Tooth disease
2005, Physical Medicine and Rehabilitation Clinics of North AmericaCitation Excerpt :A more recent gene mapping study identified a point mutation in the PMP-22 gene on mouse chromosome 11, which causes a glycine to aspartic acid substitution in a putative membrane domain of the PMP-22 protein [37]. Pathologic and nerve conduction studies have suggested that the severity of demyelination in the tr mouse is comparable to that seen in human Dejerine-Sottas disease (CMT type 3, CMT3) [13,36]. However, no human cases of demyelinating peripheral neuropathy with a gene defect homologous to that of the tr mouse have been reported.
Transgenic Models of Inherited Neuropathy
2005, Peripheral Neuropathy: 2-Volume Set with Expert Consult BasicTransgenic Models of Inherited Neuropathy
2005, Peripheral NeuropathyDialogues: Communication Between Axons and Myelinating Glia
2005, Multiple Sclerosis As A Neuronal Disease
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This work was supported by grants from the National Health and Medical Research Council of Australia, and from the Postgraduate Medical Foundation, University of Sydney.