| http://purl.uniprot.org/citations/27851749 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/27851749 | http://www.w3.org/2000/01/rdf-schema#comment | "As a deubiquitinating enzyme (DUB), the physiological substrates of ataxin-3 (ATX-3) remain elusive, which limits our understanding of its normal cellular function and that of pathogenic mechanism of spinocerebellar ataxia type 3 (SCA3). Here, we identify p53 to be a novel substrate of ATX-3. ATX-3 binds to native and polyubiquitinated p53 and deubiquitinates and stabilizes p53 by repressing its degradation through the ubiquitin (Ub)-proteasome pathway. ATX-3 deletion destabilizes p53, resulting in deficiency of p53 activity and functions, whereas ectopic expression of ATX-3 induces selective transcription/expression of p53 target genes and promotes p53-dependent apoptosis in both mammalian cells and the central nervous system of zebrafish. Furthermore, the polyglutamine (polyQ)-expanded ATX-3 retains enhanced interaction and deubiquitination catalytic activity to p53 and causes more severe p53-dependent neurodegeneration in zebrafish brains and in the substantia nigra pars compacta (SNpc) or striatum of a transgenic SCA3 mouse model. Our findings identify a novel molecular link between ATX-3 and p53-mediated cell death and provide an explanation for the direct involvement of p53 in SCA3 disease pathogenesis."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.org/dc/terms/identifier | "doi:10.1371/journal.pbio.2000733"xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/author | "Liu H."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/author | "Liu C."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/author | "Liu X."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/author | "Li X."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/author | "Ma X."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/author | "Niu Y."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/author | "Guo C."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/author | "Wang Q."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/author | "Zhu S."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/author | "Huang M."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/author | "Schmitt I."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/author | "Ning G."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/author | "Tang T.S."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/author | "Wullner U."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/date | "2016"xsd:gYear |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/name | "PLoS Biol"xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/pages | "e2000733"xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/title | "The Machado-Joseph Disease Deubiquitinase Ataxin-3 Regulates the Stability and Apoptotic Function of p53."xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://purl.uniprot.org/core/volume | "14"xsd:string |
| http://purl.uniprot.org/citations/27851749 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/27851749 |
| http://purl.uniprot.org/citations/27851749 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/27851749 |
| http://purl.uniprot.org/uniprot/#_E9Q717-mappedCitation-27851749 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/27851749 |