A new mouse model may help scientists elucidate early stages of ALS, including sporadic disease. The model, developed by Kevin Talbot and colleagues at the University of Oxford in England, exhibits key signs of ALS including progressive motor deficits and loss of muscle strength.
The mice, harboring two copies of the ALS-linked M337V human TDP-43 gene, develop symptoms at about 6 months. And by 9 to 12 months, the neuromuscular junctions began to crumble. No microglia-mediated neuroinflammation or cytoplasmic TDP-43 aggregates could be detected.
The BAC transgenic mice carry the complete human TDP-43 locus including its regulatory regions. The model is one of a growing number of mice that exhibit key features of the human disease without overexpressing the disease gene (see May 2016, November 2017, December 2017, May 2018 news; Liu et al., 2016; Devoy et al., 2017; Fratta et al., 2018).
The study is published online on October 2 in Neurobiology of Disease.
To obtain these ALS model mice for studies in your laboratory, check out this page on the Jackson Laboratory website.
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