Ontology highlight
ABSTRACT:
SUBMITTER: Ito Y
PROVIDER: S-EPMC5444917 | biostudies-literature | 2016 Aug
REPOSITORIES: biostudies-literature
Ito Yasushi Y Ofengeim Dimitry D Najafov Ayaz A Das Sudeshna S Saberi Shahram S Li Ying Y Hitomi Junichi J Zhu Hong H Chen Hongbo H Mayo Lior L Geng Jiefei J Amin Palak P DeWitt Judy Park JP Mookhtiar Adnan Kasim AK Florez Marcus M Ouchida Amanda Tomie AT Fan Jian-bing JB Pasparakis Manolis M Kelliher Michelle A MA Ravits John J Yuan Junying J
Science (New York, N.Y.) 20160801 6299
Mutations in the optineurin (OPTN) gene have been implicated in both familial and sporadic amyotrophic lateral sclerosis (ALS). However, the role of this protein in the central nervous system (CNS) and how it may contribute to ALS pathology are unclear. Here, we found that optineurin actively suppressed receptor-interacting kinase 1 (RIPK1)-dependent signaling by regulating its turnover. Loss of OPTN led to progressive dysmyelination and axonal degeneration through engagement of necroptotic mach ...[more]