Disruption of Endosomal Sorting in Schwann Cells Leads to Defective Myelination and Endosomal Abnormalities Observed in Charcot-Marie-Tooth Disease

• Published in: The Journal of neuroscience Vol. 42; no. 25; pp. 5085 - 5101
• Main Authors: McLean, John W, Wilson, Julie A, Tian, Tina, Watson, Jennifer A, VanHart, Mary, Bean, Andrew J, Scherer, Steven S, Crossman, David K, Ubogu, Eroboghene, Wilson, Scott M
• Format: Journal Article
• Language: English
• Published: United States Society for Neuroscience 22.06.2022
• Subjects: Abnormalities; Animals; Axons; Cell differentiation; Charcot-Marie-Tooth disease; Charcot-Marie-Tooth Disease - metabolism; Clonal deletion; Demyelination; Endosomal Sorting Complexes Required for Transport; Endosomes; Endosomes - metabolism; ErbB-2 protein; Genes; Inactivation; Kinases; Maturation; Mice; mRNA; Mutation; Myelination; Nerve conduction; Nerves; Peripheral Nervous System Diseases; Peripheral neuropathy; Point mutation; Protein-tyrosine kinase; Proteins; Receptor mechanisms; Receptors; RNA, Messenger; Scaffolding; Schwann cells; Schwann Cells - metabolism; Spatial distribution; Substrates; Teetering; Tyrosine; myelination; endosome; Charcot-Marie-Tooth; ESCRT; Schwann cell

Endosomal sorting plays a fundamental role in directing neural development. By altering the temporal and spatial distribution of membrane receptors, endosomes regulate signaling pathways that control the differentiation and function of neural cells. Several genes linked to inherited demyelinating pe...