News Column

Recent Findings in Amyotrophic Lateral Sclerosis Described by Researchers from University of Tokyo

January 31, 2014

By a News Reporter-Staff News Editor at Genomics & Genetics Weekly -- Investigators publish new report on Nervous System Diseases and Conditions. According to news reporting from Tokyo, Japan, by NewsRx journalists, research stated, "TDP-43 is a discriminative protein that is found as intracellular aggregations in the neurons of the cerebral cortex and spinal cord of patients with amyotrophic lateral sclerosis (ALS); however, the mechanisms of neuron loss and its relation to the aggregations are still unclear. In this study, we generated a useful model to produce TDP-43 aggregations in the motor cortex using in utero electroporation on mouse embryos."

The news correspondents obtained a quote from the research from the University of Tokyo, "The plasmids used were full-length TDP-43 and C-terminal fragments of TDP-43 (wild-type or M337V mutant) tagged with GFP. For the full-length TDP-43, both wild-type and mutant, electroporated TDP43 localized mostly in the nucleus, and though aggregations were detected in embryonic brains, they were very rarely observed at P7 and P21. In contrast, TDP-43 aggregations were generated in the brains electroporated with the C-terminal TDP-43 fragments as previously reported in in vitro experiments. TDP43 protein was distributed diffusely not only in the nucleus, but also in the cytoplasm and the inclusion bodies were ubiquitinated and included phosphorylated TDP-43, which reflects the human pathology of ALS."

According to the news reporters, the research concluded: "This model using in utero electroporation of pathogenic genes into the brain of the mouse will likely become a useful model for studying ALS and also for evaluation of agents for therapeutic purpose, and may be applicable to other neurodegenerative diseases, as well."

For more information on this research see: A unique mouse model for investigating the properties of amyotrophic lateral sclerosis-associated protein TDP-43, by in utero electroporation. Neuroscience Research, 2013;77(4):234-241. Neuroscience Research can be contacted at: Elsevier Ireland Ltd, Elsevier House, Brookvale Plaza, East Park Shannon, Co, Clare, 00000, Ireland. (Elsevier -; Neuroscience Research -

Our news journalists report that additional information may be obtained by contacting M. Akamatsu, University of Tokyo, Grad Sch Med, Center Dis Biol & Integrat Med, Div Clin Biotechnol, Tokyo 1138654, Japan. Additional authors for this research include H. Takuma, T. Yamashita, T. Okada, K. Keino-Masu, K. Ishii, S. Kwak, M. Masu and A. Tamaoka (see also Nervous System Diseases and Conditions).

Keywords for this news article include: Asia, Tokyo, Japan, Genetics, Neurology, Therapeutics, Motor Neuron Disease, Spinal Cord Diseases, Neuromuscular Diseases, TDP-43 Proteinopathies, Proteostasis Deficiencies, Neurodegenerative Diseases, Amyotrophic Lateral Sclerosis, Central Nervous System Diseases, Nutritional and Metabolic Diseases

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Source: Genomics & Genetics Weekly

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