Adenoviral-mediated mouse model of motor impairment in distal spinal muscular atrophy type V

Ah Jung Seo, Byung Sun Park, Na Young Jeong, Doyen Kim, Sunghoon Kim, Chan Park, Junyang Jung, Youngbuhm Huh

Research output: Contribution to journalArticlepeer-review

1 Citation (Scopus)


Distal spinal muscular atrophy type V (dSMA-V) is a hereditary neurodegenerative axonal neuropathy and a glycyl-tRNA synthetase (GARS)-associated neuropathy that is caused by a mutation in GARS. Herein, we report a new GARS-associated neuropathy mouse model using an adenovirus vector system equipped with a neuron-specific promoter. In this model, we showed an increased number of activated microglia around the L129P mutant-expressing motor neuron cell bodies and the increased nerve injury signal in L129P mutant-expressing motor neuron cell bodies. In addition, we found that the mouse model exhibited impaired motor function caused by the L129P mutant. We propose that the neuroinflammation may be associated with motor neuron impairment in GARS-associated neuropathies.

Original languageEnglish
Pages (from-to)311-317
Number of pages7
JournalAnimal Cells and Systems
Issue number5
Publication statusPublished - 2014 Sept 30

Bibliographical note

Funding Information:
We thank Dr Doyeun Kim (Medicinal Bioconvergence Research Center, Seoul National University, Seoul, Korea) for valuable discussion and Dr Paul Schimmel (Scripps Research Institute, La Jolla, CA) for kindly providing the WT and L129P mutant GARS clones. This work was supported by the National Research Foundation (NRF) of Korea grant funded by Korean Government (MEST) (No. 2011-0030072).

Publisher Copyright:
© 2014, © 2014 Korean Society for Integrative Biology.

All Science Journal Classification (ASJC) codes

  • Animal Science and Zoology
  • Biochemistry, Genetics and Molecular Biology(all)


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