ASK1 is essential for endoplasmic reticulum stress-induced …
2002年6月1日 · In the present study, we investigated the role of ASK1 in the pathogenesis of polyQ diseases. PolyQ fragments with pathogenic repeat length inhibited proteasomal activity …
In Situ Architecture and Cellular Interactions of PolyQ Inclusions
2017年9月21日 · IRE1 plays an essential role in ER stress-mediated aggregation of mutant huntingtin via the inhibition of autophagy flux
ASK1 is essential for endoplasmic reticulum stress-induced ... - PubMed
2002年6月1日 · Here, we show the following: (1) polyQ with pathogenic repeat length triggers ER stress through proteasomal dysfunction; (2) ER stress activates ASK 1 through formation of an …
The Role of IRE1 Signaling in the Central Nervous System Diseases
The mutation responsible for HD leads to an abnormally long polyglutamine (polyQ) expansion in the huntingtin (Htt) protein, which confers one or more toxic functions to mutant Htt leading to …
Mammalian endoplasmic reticulum stress sensor IRE1 signals by ... - PNAS
2010年7月20日 · The ER-resident transmembrane kinase-endoribonuclease inositol-requiring enzyme 1 (IRE1) is the best characterized UPR signal transduction molecule. In yeast, Ire1 …
Impact of ER Stress and ER-Mitochondrial Crosstalk in …
This study began a new concept that linked ER stress and HD pathology. A subsequent study investigating the cause of cell death in PC12 and related neuronal cell lines harboring PolyQ …
Signaling Pathways from the Endoplasmic Reticulum and Their …
In mammals, the UPR signaling pathway is initiated by three ER membrane-associated sensors: activating transcription factor-6 (ATF6), inositol-requiring transmembrane …
The functions of IRE1α in neurodegenerative diseases
2022年12月1日 · Expanded polyglutamine (PolyQ) induces ER stress in neurons and activates IRE1α, which forms a protein complex with TRAF2 and ASK1 (IRE1α-TRAF2-ASK1) and …
Activated IRE1 by polyQ induced the TRAF2–ASK1 complex formation that led to JNK activation. We show that primary neu-rons derived from ASK1−/− mice were resistant to ER stress-, …
Molecular functions of ASK family in diseases caused by stress …
2020年12月30日 · We showed that expansion of the polyQ sequence causes ER stress via proteasome dysfunction, accompanying ASK1 activation via formation of the …