Ubiquilin-2 (IPR028430)

Short name: Ubiquilin-2

Overlapping homologous superfamilies


Family relationships

  • Ubiquilin (IPR015496)
    • Ubiquilin-2 (IPR028430)


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-2.

Ubiquilin-2 increases the half-life of proteins destined to be degraded by the proteasome [PMID: 10983987]. 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 [PMID: 21857683, PMID: 22892309, PMID: 22717235, PMID: 22560112].

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.