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, 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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