Schwann cell deletion of Tumor Susceptibility Gene 101 (Tsg101) in mice results in severe peripheral neuropathy

Myelinating Schwann cells are particularly susceptible to defects in endosomal trafficking. TSG101 is a component of the endosomal trafficking machinery that mediates the sorting of ubiquitinated receptors into multivesicular bodies. We previously demonstrated that deleting <i>Tsg101</i> from mouse oligodendrocytes in the central nervous system causes rapid onset de/dys-myelination and vacuolation of white matter, suggesting an important role for TSG101-dependent trafficking in myelination. Here, we show that TSG101 is also required for normal myelination in the peripheral nervous system.