A new mouse model of Charcot-Marie-Tooth 2J neuropathy replicates human axonopathy and suggest alteration in axo-glia communication - Shackleford, Ghjuvan’Ghjacumu; Marziali, Leandro N.; Sasaki, Yo; Claessens, Anke; Ferri, Cinzia; Weinstock, Nadav I.; Rossor, Alexander M.; Silvestri, Nicholas J.; Wilson, Emma R.; Hurley, Edward; Kidd, Grahame J.; Manohar, Senthilvelan; et al. (PLoS genetics, 2022)
Keywords: Action potential; Age; Animal models; Animal models in research; Animals; Axon guidance; Axonal transport; Axons; Axons - metabolism; Biology and Life Sciences; Cell interactions; Charcot-Marie-Tooth disease; Charcot-Marie-Tooth Disease - genetics; Communication; Degeneration; Demyelination; Development and progression; Disease Models, Animal; Gap junctions; Genotype & phenotype; Health aspects; Hearing loss; Humans; Medicine and Health Sciences; Methionine; Mice; Mice, Knockout; Mitochondria; Motor task performance; Mutants; Mutation; Myelin P0 protein; Myelin proteolipid protein; Myelin Sheath - genetics; Myelin Sheath - metabolism; Myelin-associated glycoprotein; Myelination; Nerve conduction; Nervous system; Neurodegeneration; Neuroglia; Neurological research; Neuronal-glial interactions; Neuropathy; Peripheral neuropathy; Physiological aspects; Proteins; Research and Analysis Methods; Schwann cells; Threonine; United States