| http://purl.uniprot.org/citations/24244371 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/24244371 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/24244371 | http://www.w3.org/2000/01/rdf-schema#comment | "Senataxin is a large 303 kDa protein linked to neuron survival, as recessive mutations cause Ataxia with Oculomotor Apraxia type 2 (AOA2), and dominant mutations cause amyotrophic lateral sclerosis type 4 (ALS4). Senataxin contains an amino-terminal protein-interaction domain and a carboxy-terminal DNA/RNA helicase domain. In this study, we focused upon the common ALS4 mutation, L389S, by performing yeast two-hybrid screens of a human brain expression library with control senataxin or L389S senataxin as bait. Interacting clones identified from the two screens were collated, and redundant hits and false positives subtracted to yield a set of 13 protein interactors. Among these hits, we discovered a highly specific and reproducible interaction of L389S senataxin with a peptide encoded by the antisense sequence of a brain-specific non-coding RNA, known as BCYRN1. We further found that L389S senataxin interacts with other proteins containing regions of conserved homology with the BCYRN1 reverse complement-encoded peptide, suggesting that such aberrant protein interactions may contribute to L389S ALS4 disease pathogenesis. As the yeast two-hybrid screen also demonstrated senataxin self-association, we confirmed senataxin dimerization via its amino-terminal binding domain and determined that the L389S mutation does not abrogate senataxin self-association. Finally, based upon detection of interactions between senataxin and ubiquitin-SUMO pathway modification enzymes, we examined senataxin for the presence of ubiquitin and SUMO monomers, and observed this post-translational modification. Our senataxin protein interaction study reveals a number of features of senataxin biology that shed light on senataxin normal function and likely on senataxin molecular pathology in ALS4."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.org/dc/terms/identifier | "doi:10.1371/journal.pone.0078837"xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.org/dc/terms/identifier | "doi:10.1371/journal.pone.0078837"xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Chen Y."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Chen Y."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Fields S."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Fields S."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Lo R.S."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Lo R.S."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Bennett C.L."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Bennett C.L."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "La Spada A.R."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "La Spada A.R."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Vignali M."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Vignali M."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Mason A.G."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Mason A.G."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Huq Saifee N.P."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Huq Saifee N.P."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Unal A."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/author | "Unal A."xsd:string |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/date | "2013"xsd:gYear |
| http://purl.uniprot.org/citations/24244371 | http://purl.uniprot.org/core/date | "2013"xsd:gYear |