| http://purl.uniprot.org/citations/14597671 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/14597671 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/14597671 | http://www.w3.org/2000/01/rdf-schema#comment | "Molecular misreading of the ubiquitin-B (UBB) gene results in a dinucleotide deletion in UBB mRNA. The resulting mutant protein, UBB+1, accumulates in the neuropathological hallmarks of Alzheimer disease. In vitro, UBB+1 inhibits proteasomal proteolysis, although it is also an ubiquitin fusion degradation substrate for the proteasome. Using the ligase chain reaction to detect dinucleotide deletions, we report here that UBB+1 transcripts are present in each neurodegenerative disease studied (tauo- and synucleinopathies) and even in control brain samples. In contrast to UBB+1 transcripts, UBB+1 protein accumulation in the ubiquitin-containing neuropathological hallmarks is restricted to the tauopathies such as Pick disease, frontotemporal dementia, progressive supranuclear palsy, and argyrophilic grain disease. Remarkably, UBB+1 protein is not detected in the major forms of synucleinopathies (Lewy body disease and multiple system atrophy). The neurologically intact brain can cope with UBB+1 as lentivirally delivered UBB+1 protein is rapidly degraded in rat hippocampus, whereas the K29,48R mutant of UBB+1, which is not ubiquitinated, is abundantly expressed. The finding that UBB+1 protein only accumulates in tauopathies thus implies that the ubiquitin-proteasome system is impaired specifically in this group of neurodegenerative diseases and not in synucleinopathies and that the presence of UBB+1 protein reports proteasomal dysfunction in the brain."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.org/dc/terms/identifier | "doi:10.1096/fj.03-0205com"xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.org/dc/terms/identifier | "doi:10.1096/fj.03-0205com"xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Fischer D.F."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Fischer D.F."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Van Dijk R."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Van Dijk R."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Kamphorst W."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Kamphorst W."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "De Vos R.A."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "De Vos R.A."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "De Vrij F.M."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "De Vrij F.M."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Hobo B."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Hobo B."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Hol E.M."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Hol E.M."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Proper E.A."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Proper E.A."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Sluijs J.A."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Sluijs J.A."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Sonnemans M.A."xsd:string |
| http://purl.uniprot.org/citations/14597671 | http://purl.uniprot.org/core/author | "Sonnemans M.A."xsd:string |