000007580 001__ 7580 000007580 005__ 20231129124939.0 000007580 0247_ $$2DOI$$a10.6083/js956g337 000007580 037__ $$aETD 000007580 245__ $$aDistinct roles for the endosomal regulators Mtmr5 and Mtmr13 in axon radial sorting and myelination 000007580 260__ $$bOregon Health and Science University 000007580 269__ $$a2019 000007580 336__ $$aDissertation 000007580 502__ $$bPh.D. 000007580 520__ $$aNearly half of the proteins linked to demyelinating subtypes of Charcot-Marie-Tooth disease regulate endo-lysosomal trafficking. Most of these proteins are expressed in all human tissues, yet defects caused by loss-of-function mutations are largely isolated to the peripheral nervous system. This observation suggests that myelinating Schwann cells are particularly vulnerable to endosomal trafficking defects, yet the molecular mechanisms that orchestrate this cell-specific vulnerability remain unclear. We hypothesize that endosomal trafficking provides the specific control needed to regulate pro-myelination signals and coordinate cytoskeletal rearrangements during axon sorting and myelination. All cells require the trafficking of membrane receptors, but the work described within this dissertation suggests that Schwann cells rely heavily on endo-lysosomal membrane trafficking for all aspects of their biology. Highlighting why changes in phosphoinositide hydrolysis and endosomal trafficking cause peripheral neuropathy. 000007580 542__ $$fIn copyright - single owner 000007580 650__ $$aSchwann Cells$$025781 000007580 650__ $$aEndosomal Sorting Complexes Required for Transport$$038727 000007580 6531_ $$amyelin 000007580 6531_ $$aphosphoinositides 000007580 6531_ $$acharcot marie tooth disease 000007580 6531_ $$amyotubularin 000007580 691__ $$aSchool of Medicine$$041369 000007580 7001_ $$aMammel, Anna E. 000007580 8564_ $$9609a499b-9c75-46bb-af9a-8cd1cdf9ab44$$s4990314$$uhttps://digitalcollections.ohsu.edu/record/7580/files/mammel.anna.2019.pdf 000007580 905__ $$a/rest/prod/js/95/6g/33/js956g337 000007580 909CO $$ooai:digitalcollections.ohsu.edu:7580$$pstudent-work 000007580 980__ $$aTheses and Dissertations