| Primary Identifier | IPR028430 | Type | Family |
| Short Name | Ubiquilin-1/2 |
| description | Ubiquilins, also known as proteins linking IAP to cytoskeleton (PLICs), play a role in protein degradation through interactions with their N-terminal ubiquitin-like (UBL) and C-terminal ubiquitin-associated (UBA) domains, which associate with proteasomes and ubiquitin ligases. Humans and mice each possess four ubiquilin proteins. This entry includes ubiquilin-1 and 2.Ubiquilin-1 (also known as PLIC-1) is an ubiquitin-like protein that regulates the stability of various proteins, including amyloid precursor protein, Trif and presenilin [, ]. Ubiquilin-1 enhances the stability of GABA(A) receptors within the secretory pathway and promotes their insertion into the neuronal plasma membrane []. It inhibits TLR3-Trif signalling (an antiviral pathway) []. It interacts with mTOR, which is a protein kinase that controls cell cycle progression and cell growth through regulation of translation, transcription, membrane traffic and protein degradation [].Ubiquilin-2 increases the half-life of proteins destined to be degraded by the proteasome []. Mutations in the ubiquilin-2 gene cause amyotrophic lateral sclerosis 15, with or without frontotemporal dementia (ALS15), which is a neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis [, , , ]. |
