Ubiquilin-1 and protein quality control in alzheimer disease

Amina El Ayadi, Emily S. Stieren, José M. Barral, Darren Boehning

    Research output: Contribution to journalArticle

    9 Citations (Scopus)

    Abstract

    Single nucleotide polymorphisms in the ubiquilin-1 gene may confer risk for late-onset Alzheimer disease (AD). We have shown previously that ubiquilin-1 functions as a molecular chaperone for the amyloid precursor protein (APP) and that protein levels of ubiquilin-1 are decreased in the brains of AD patients. We have recently found that ubiquilin-1 regulates APP trafficking and subsequent secretase processing by stimulating non-degradative ubiquitination of a single lysine residue in the cytosolic domain of APP. Thus, ubiquilin- 1 plays a central role in regulating APP biosynthesis, trafficking and ultimately toxicity. As ubiquilin-1 and other ubiquilin family members have now been implicated in the pathogenesis of numerous neurodegenerative diseases, these findings provide mechanistic insights into the central role of ubiquilin proteins in maintaining neuronal proteostasis.

    Original languageEnglish (US)
    Pages (from-to)164-169
    Number of pages6
    JournalPrion
    Volume7
    Issue number2
    DOIs
    StatePublished - Mar 2013

    Fingerprint

    Amyloid beta-Protein Precursor
    Protein Transport
    Amyloid
    Quality Control
    Quality control
    Alzheimer Disease
    Amyloid Precursor Protein Secretases
    Molecular Chaperones
    Ubiquitination
    Brain Diseases
    Protein Biosynthesis
    Neurodegenerative Diseases
    Lysine
    Single Nucleotide Polymorphism
    Proteins
    Neurodegenerative diseases
    Biosynthesis
    Polymorphism
    Genes
    Toxicity

    Keywords

    • Alzheimer disease
    • Amyloid precursor protein
    • Molecular chaperone
    • Trafficking
    • Ubiquilin-1
    • Ubiquitination

    ASJC Scopus subject areas

    • Biochemistry
    • Cell Biology
    • Infectious Diseases
    • Cellular and Molecular Neuroscience

    Cite this

    Ayadi, A. E., Stieren, E. S., Barral, J. M., & Boehning, D. (2013). Ubiquilin-1 and protein quality control in alzheimer disease. Prion, 7(2), 164-169. https://doi.org/10.4161/pri.23711

    Ubiquilin-1 and protein quality control in alzheimer disease. / Ayadi, Amina El; Stieren, Emily S.; Barral, José M.; Boehning, Darren.

    In: Prion, Vol. 7, No. 2, 03.2013, p. 164-169.

    Research output: Contribution to journalArticle

    Ayadi, AE, Stieren, ES, Barral, JM & Boehning, D 2013, 'Ubiquilin-1 and protein quality control in alzheimer disease', Prion, vol. 7, no. 2, pp. 164-169. https://doi.org/10.4161/pri.23711
    Ayadi AE, Stieren ES, Barral JM, Boehning D. Ubiquilin-1 and protein quality control in alzheimer disease. Prion. 2013 Mar;7(2):164-169. https://doi.org/10.4161/pri.23711
    Ayadi, Amina El ; Stieren, Emily S. ; Barral, José M. ; Boehning, Darren. / Ubiquilin-1 and protein quality control in alzheimer disease. In: Prion. 2013 ; Vol. 7, No. 2. pp. 164-169.
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