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

## TL;DR

Deleting a specific gene in nerve cells causes severe nerve damage in mice, highlighting its role in healthy nerve function.

## Contribution

This study shows that TSG101 is essential for myelination in the peripheral nervous system.

## Key findings

- Deleting Tsg101 in Schwann cells causes severe peripheral neuropathy in mice.
- TSG101 is required for normal myelination in the peripheral nervous system.
- The gene plays a key role in endosomal trafficking in Schwann cells.

## Abstract

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
Tsg101
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.