Complement components are upregulated and correlate with disease progression in the TDP-43Q331K mouse model of amyotrophic lateral sclerosis.
John D LeeSamantha C LevinEmily F WillisRui LiTrent M WoodruffPeter G NoakesPublished in: Journal of neuroinflammation (2018)
These results indicate that similar to SOD1 transgenic animals, local complement activation and increased expression of C5aR1 may contribute to motor neuron death and neuromuscular junction denervation in the TDP-43Q331K mouse ALS model. This further validates C5aR1 as a potential therapeutic target for ALS.