Ubiquilin 2: A component of the ubiquitin-proteasome system with an emerging role in neurodegeneration

Katharine Y. Zhang, Shu Yang, Sadaf T. Warraich, Ian P. Blair*

*Corresponding author for this work

    Research output: Contribution to journalArticlepeer-review

    42 Citations (Scopus)


    Ubiquilin 2, which is encoded by the UBQLN2 gene, plays a critical role in protein clearance pathways including the ubiquitin-proteasome system and autophagy. Ubiquilin 2 physically associates with ubiquitin ligases and proteasomes to mediate protein degradation. It also plays a role in the regulation of cell signalling and cell cycle progression, and association with cytoskeletal elements. Recent studies have revealed that ubiquilin 2 also plays a pathogenic role in neurodegenerative disease, including amyotrophic lateral sclerosis (ALS), and ALS-frontotemporal dementia (ALS-FTD). Rare UBQLN2 mutations cause a small subset of ALS and ALS-FTD cases. More widespread is the presence of ubiquilin 2 positive inclusions in the affected neurons of some familial and sporadic ALS and ALS-FTD patients. These discoveries have led to the hypothesis that perturbation in protein clearance, mediated by ubiquilin 2, is an important pathogenic mechanism in neurodegeneration.

    Original languageEnglish
    Pages (from-to)123-126
    Number of pages4
    JournalInternational Journal of Biochemistry and Cell Biology
    Publication statusPublished - May 2014


    • Amyotrophic lateral sclerosis
    • Autophagy
    • Dementia
    • Ubiquilin 2
    • Ubiquitin-proteasome system


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