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Identification of novel neuroprotective molecule effective in ALS mouse models NFIL3 plays a neuroprotective role in neurons

January 27, 2014

Amyotrophic lateral sclerosis (ALS) is a progressive, fatal and untreatable neurodegenerative disease characterized by the loss of motor neurons, muscle wasting and weakness. Death due to respiratory muscle paralysis usually results within a few years. While numerous studies aim to attenuate neurodegeneration in ALS, enhancement of defense mechanisms intrinsic to neurons remains a potential strategy to counteract neuron death in ALS. Thus, uncovering novel neuroprotective molecules within neurons could lead to the development of therapeutic approaches for ALS and related neurodegenerative diseases.

© Kamon Sanada. NFIL3 overexpression in ALS mice delays phenotypic disease onset in ALS mice. (Left) An ALS mouse at about 260-days of age. The mouse shows denervation-induced muscle atrophy. (Right) An age-matched ALS mouse with neuronal overexpression of NFIL3. NFIL3 overexpression attenuates muscle atrophy.

The research group of Associate Professor Kamon Sanada at the University of Tokyo Graduate School of Science’s Molecular Genetics Research Laboratory, collaborating with the research group of Professor Yoshitaka Fukuda at the Department of Biological Chemistry, has established that the NFIL3 protein plays a neuroprotective role within neurons. Transgenic neuronal expression of NFIL3 in mice that demonstrate ALS-like symptoms such as muscle wastage (ALS model mice) delays disease onset and attenuates motor axon and neuron degeneration.

These results suggest that NFIL3 confers neuroprotection in models of ALS and constitute a potential therapeutic target for neurodegeneration in ALS and related neurodegenerative diseases.

Paper

So-ichi Tamai, Keisuke Imaizumi, Nobuhiro Kurabayashi, Minh Dang Nguyen, Takaya Abe, Masatoshi Inoue, Yoshitaka Fukada, and Kamon Sanada,
“Neuroprotective role of the basic leucine zipper transcription factor NFIL3 in models of amyotrophic lateral sclerosis”,
The Journal of Biological Chemistry, Jan 17, 2014, doi: 10.1074/jbc.M113.524389.
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Graduate School of Science

Department of Biophysics and Biochemistry, Graduate School of Science

Molecular Genetic Research Laboratory, Department of Biophysics and Biochemistry, Graduate School of Science

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