However, the presence of a mutant form of ubiquitin B, the UBB+1 protein, in disease-specific aggregates (e.g., neurofibrillary tangles and intranuclear inclusions) of patients suffering from tauopathies and polyglutamine diseases [7, 10] may point to a causative role of UPS dysfunction in these diseases rather than being a consequence. This evidence concerns the gene UBB and tauopathy.