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

Ju Hwang Park, Hang Soo Park, Sunghoi Hong, Seong Man Kang

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

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
Pages (from-to)e276
JournalExperimental & molecular medicine
Volume48
Issue number12
DOIs
Publication statusPublished - 2016 Dec 9

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Induced Pluripotent Stem Cells
Amyotrophic Lateral Sclerosis
Motor Neurons
Stem cells
Neurons
Neurodegenerative diseases
Cell death
Neurodegenerative Diseases
Superoxide Dismutase
Brain
Genes
Cells
Germ Layers
Neurites
Transgenic Mice
Cell Survival
Spinal Cord
Cell Death
Technology
Mutation

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Medicine
  • Molecular Biology
  • Clinical Biochemistry

Cite this

Motor neurons derived from ALS-related mouse iPS cells recapitulate pathological features of ALS. / Park, Ju Hwang; Park, Hang Soo; Hong, Sunghoi; Kang, Seong Man.

In: Experimental & molecular medicine, Vol. 48, No. 12, 09.12.2016, p. e276.

Research output: Contribution to journalArticle

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