Article
Linear ubiquitination is involved in the pathogenesis of optineurin-associated amyotrophic lateral sclerosis.
Nature communications - 24 Aug 2016
Nakazawa Seshiru, Oikawa Daisuke, Ishii Ryohei, Ayaki Takashi, Takahashi Hirotaka, Takeda Hiroyuki, Ishitani Ryuichiro, Kamei Kiyoko, Takeyoshi Izumi, Kawakami Hideshi, Iwai Kazuhiro, Hatada Izuho, Sawasaki Tatsuya, Ito Hidefumi, Nureki Osamu, Tokunaga Fuminori
Abstract excerpt
Optineurin (OPTN) mutations cause neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and glaucoma. Although the ALS-associated E478G mutation in the UBAN domain of OPTN reportedly abolishes its NF-κB suppressive activity, the precise molecular basis in ALS pathogenesis still remains unclear. Here we report that the OPTN-UBAN domain is crucial for NF-κB suppression. Our crystal structure...
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