• Publications
  • Influence
Mutant ubiquitin decreases amyloid β plaque formation in a transgenic mouse model of Alzheimer’s disease
The mutant ubiquitin UBB(+1) is a substrate as well as an inhibitor of the ubiquitin-proteasome system (UPS) and accumulates in the neuropathological hallmarks of Alzheimer's disease (AD). A role forExpand
  • 27
  • 3
Misframed ubiquitin and impaired protein quality control: an early event in Alzheimer’s disease
Amyloid β (Aβ) plaque formation is a prominent cellular hallmark of Alzheimer’s disease (AD). To date, immunization trials in AD patients have not been effective in terms of curing or amelioratingExpand
  • 25
  • 2
  • Open Access
Accumulation of Basic Amino Acids at Mitochondria Dictates the Cytotoxicity of Aberrant Ubiquitin
Summary Neuronal accumulation of UBB+1, a frameshift variant of ubiquitin B, is a hallmark of Alzheimer’s disease (AD). How UBB+1 contributes to neuronal dysfunction remains elusive. Here, we showExpand
  • 42
Long-term proteasomal inhibition in transgenic mice by UBB+1 expression results in dysfunction of central respiration control reminiscent of brainstem neuropathology in Alzheimer patients
Aging and neurodegeneration are often accompanied by a functionally impaired ubiquitin–proteasome system (UPS). In tauopathies and polyglutamine diseases, a mutant form of ubiquitin B (UBB+1)Expand
  • 22
Localization of mutant ubiquitin in the brain of a transgenic mouse line with proteasomal inhibition and its validation at specific sites in Alzheimer's disease
Loss of protein quality control by the ubiquitin-proteasome system (UPS) during aging is one of the processes putatively contributing to cellular stress and Alzheimer's disease (AD) pathogenesis.Expand
  • 9
  • Open Access
Modeling non-hereditary mechanisms of Alzheimer disease during apoptosis in yeast
Impaired protein degradation and mitochondrial dysfunction are believed to contribute to neurodegenerative disorders, including Alzheimer disease (AD). In patients suffering from non-hereditary AD,Expand
  • 8
  • Open Access
Selective Transgenic Expression of Mutant Ubiquitin in Purkinje Cell Stripes in the Cerebellum
The ubiquitin-proteasome system (UPS) is one of the major mechanisms for protein breakdown in cells, targeting proteins for degradation by enzymatically conjugating them to ubiquitin molecules.Expand
  • 1