| Post Translational Modifications | Phosphorylated in vitro by MAPK8/JNK1 on Ser-739. Mutant ATN1 sequences with expanded poly-Gln (polyQ) traits are more slowly phosphorylated. Proteolytically cleaved, probably in the nucleus, to produce two C-terminal fragments of 140 kDa (F1) and 125 kDa (F2) each containing poly-Gln (polyQ) tracts. F2 is produced by cleavage by caspases and is exported into the cytoplasm. In vitro, cleavage increases with an increase in the number of polyQ tracts. C-terminal proteolytic products appear to be the cause of cell toxicity. In vitro cleavage at Asp-109. |
| Function | Transcriptional corepressor. Recruits NR2E1 to repress transcription. Promotes vascular smooth cell (VSMC) migration and orientation. Corepressor of MTG8 transcriptional repression. Has some intrinsic repression activity which is independent of the number of poly-Gln (polyQ) repeats. |
| Protein Name | Atrophin-1Dentatorubral-Pallidoluysian Atrophy Protein |
| Database Links | Reactome: R-HSA-8943724 |
| Cellular Localisation | NucleusCytoplasmPerinuclear RegionCell JunctionShuttles Between Nucleus And CytoplasmColocalizes With Fat1 In The Perinuclear AreaAt Cell-Cell Junctions And Leading Edges Of CellsColocalizes With Mtg8 In Discrete Nuclear DotsProteolytic Fragment F1 Appears To Remain In NucleusFragment F2 Is Exported Into The CytoplasmFragment F2 From Mutant Sequences With Longer Poly-Gln (Polyq) Tracts Are Additionally Located To The Cytoplasmic Membrane And To Certain Organelles |
| Alternative Antibody Names | Anti-Atrophin-1 antibodyAnti-Dentatorubral-Pallidoluysian Atrophy Protein antibodyAnti-ATN1 antibodyAnti-D12S755E antibodyAnti-DRPLA antibody |
Information sourced from Uniprot.org
