|
KMID : 1161520150190060359
|
|
Animal Cells and Systems
2015 Volume.19 No. 6 p.359 ~ p.364
|
|
|
Novel animal models of GARS-associated neuropathy by overexpression of mutant GARS using an adenoviral vector
|
|
Jeong Na-Young
Song In-Ok
Um Hyeong-Seok
Jung Jun-Yang
Huh Young-Buhm
|
|
|
Abstract
|
|
|
|
Glycyl-tRNA synthetase (GARS)-associated neuropathy caused by a mutation in GARS is a hereditary axonal neuropathy involving motor functional impairment. In this review, we describe a novel GARS-associated mouse neuropathy model produced using an adenovirus vector system containing a neuron-specific promoter. These adenovirus vector-mediated animal models are based on GARS mutations and have been generated by viral delivery of GARS proteins to the long peripheral axons of mice. Using the highly efficient adenoviral vector system, the overexpression of mutated GARS proteins in animals is sufficient to induce the same phenotypes seen in patients, such as neuropathic pain and motor function impairment. Adenoviral vector-mediated animal models have been used to demonstrate the cellular distribution patterns of GARS mutants and their induced molecular changes. In addition, the GARS-associated neuropathy model has demonstrated that neuroinflammation mediated by microglial activation is a disease phenotype-causing factor. Thus, these adenoviral vector-mediated animal models provide valuable insights into GARS-associated axonopathy and may contribute to the development of therapeutic approaches.
|
|
|
KEYWORD
|
|
|
Adenovirus, neurodegeneration, Charcot-Marie-Tooth type 2D, distal spinal muscular atrophy type V, motor impairment, neuropathic pain
|
|
|
FullTexts / Linksout information
|
|
|
|
|
|
Listed journal information
|
|
 
|