Mayumi Akizuki, Hirofumi Yamashita, Kengo Uemura, Hirofumi Maruyama, Hideshi Kawakami, Hidefumi Ito and Ryosuke Takahashi Optineurin suppression causes neuronal cell death via NF-κB pathway Journal of Neurochemistry 126
Among the genes for typical amyotrophic lateral sclerosis (ALS) phenotypes, optineurin (OPTN) is virtually the only gene in which a loss-of-function mutation is considered as the principal disease mechanism. We found that OPTN knockdown induced neuronal cell death via NF-κB activation. Furthermore, proapoptotic molecules such as p53 and Bax representing downstream targets of NF-κB are suggested to be involved in neuronal death.
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