Motor neurons derived from ALS-related mouse iPS cells recapitulate pathological features of ALS

Ju Hwang Park, Hang Soo Park, Sunghoi Hong, Seongman Kang

Research output: Contribution to journalArticlepeer-review

15 Citations (Scopus)


Amyotrophic lateral sclerosis (ALS) is a late-onset progressive neurodegenerative disease characterized by the loss of motor neurons in the spinal cord and brain. Mutations in Cu/Zn superoxide dismutase 1 (SOD1) are known to induce ALS. Although many research models have been developed, the exact pathological mechanism of ALS remains unknown. The recently developed induced pluripotent stem (iPS) cell technology is expected to illuminate the pathological mechanisms and new means of treatment for neurodegenerative diseases. To determine the pathological mechanism of ALS, we generated mouse iPS (miPS) cells from experimental ALS transgenic mice and control mice and characterized the cells using molecular biological methods. The generated miPS cells expressed many pluripotent genes and differentiated into three germ layers in vitro and in vivo. Motor neurons derived from ALS-related miPS cells recapitulated the pathological features of ALS. The ALS-model motor neurons showed SOD1 aggregates, as well as decreased cell survival rate and neurite length compared with wild-type motor neurons. Our study will be helpful in revealing the mechanism of motor neuronal cell death in ALS.

Original languageEnglish
Article numbere276
JournalExperimental and Molecular Medicine
Issue number12
Publication statusPublished - 2016 Dec

Bibliographical note

Funding Information:
This work was supported by the Korea Healthcare Technology R&D Project, Ministry for Health, Welfare & Family Affairs, Republic of Korea (no. A120340), a grant from the National R&D Program for Cancer Control, the Ministry of Health & Welfare, Republic of Korea (no. 1320010), the National Research Foundation of Korea Grants, the Ministry of Science, ICT and Future Planning, Republic of Korea (NRF-2015R1A4A1041919) and the National Research Foundation of Korea (NRF) grant (MEST) (NRF-2015R1A2A2A01003516).

Publisher Copyright:
© 2016 KSBMB. All rights reserved.

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Medicine
  • Molecular Biology
  • Clinical Biochemistry


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