CUL2-mediated clearance of misfolded TDP-43 is paradoxically affected by VHL in oligodendrocytes in ALS

The molecular machinery responsible for cytosolic accumulation of misfolded TDP-43 in amyotrophic lateral sclerosis (ALS) remains elusive. Here we identified a cullin-2 (CUL2) RING complex as a novel ubiquitin ligase for fragmented forms of TDP-43. The von Hippel Lindau protein (VHL), a substrate bi...

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Pubblicato in:Sci Rep
Autori principali: Uchida, Tsukasa, Tamaki, Yoshitaka, Ayaki, Takashi, Shodai, Akemi, Kaji, Seiji, Morimura, Toshifumi, Banno, Yoshinori, Nishitsuji, Kazuchika, Sakashita, Naomi, Maki, Takakuni, Yamashita, Hirofumi, Ito, Hidefumi, Takahashi, Ryosuke, Urushitani, Makoto
Natura: Artigo
Lingua:Inglês
Pubblicazione: Nature Publishing Group 2016
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Article
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC4707540/
https://ncbi.nlm.nih.gov/pubmed/26751167
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/srep19118
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