ALS-FTLD associated mutations of SQSTM1 impact on Keap1-Nrf2 signalling
详细信息 查看全文
- 作者：Alice Goodea ; alice.goode@nottingham.ac.uk" class="auth_mail" title="E-mail the corresponding author ; Sarah Reab ; c ; Melanie Sultanab ; c ; Barry Shawa ; Mark S. Searled ; Robert Layfielda
- 关键词：ALS ; Amyotrophic lateral sclerosis ; ARE ; Antioxidant response element ; FTLD ; Frontotemporal lobar degeneration ; IKKβ ; Inhibitor of nuclear factor kappa-B kinase subunit beta ; Keap1 ; Kelch-like ECH-associated protein 1 ; KIR ; Keap1-interacting region ; LIR ; LC3-interacting region ; mTORC1 ; Mammalian target of rapamycin complex 1 ; NFE2L2 ; Nuclear factor erythroid 2-like 2 ; NQO1 ; NAD(P)H dehydrogenase ; Quinone 1 ; Nrf2 ; Nuclear erythroid 2-related factor 2 ; PDB ; Paget's disease of bone ; RBM45 ; RNA bind
- 刊名：Molecular and Cellular Neuroscience
- 出版年：2016
- 出版时间：October 2016
- 年：2016
- 卷：76
- 期：Complete
- 页码：52-58
- 全文大小：701 K
文摘
- •
ALS-FTLD associated KIR mutations of SQSTM1/p62 disrupt Keap1 binding.
- •
KIR mutants of SQSTM1/p62 are unable to activate Nrf2 signalling in reporter assays.
- •
Some SQSTM1 mutations may contribute to ALS-FTLD through an aberrant antioxidant stress response.