Charcot-Marie-Tooth disease type 2D is a hereditary axonal and glycyl-tRNA 
synthetase (GARS)-associated neuropathy that is caused by a mutation in GARS. 
Here, we report a novel GARS-associated mouse neuropathy model using an 
adenoviral vector system that contains a neuronal-specific promoter. In this 
model, we found that wild-type GARS is distributed to peripheral axons, dorsal 
root ganglion (DRG) cell bodies, central axon terminals, and motor neuron cell 
bodies. In contrast, GARS containing a G240R mutation was localized in DRG and 
motor neuron cell bodies, but not axonal regions, in vivo. Thus, our data 
suggest that the disease-causing G240R mutation may result in a distribution 
defect of GARS in peripheral nerves in vivo. Furthermore, a distributional 
defect may be associated with axonal degradation in GARS-associated 
neuropathies.