| Post Translational Modifications | Phosphorylated by ATM at Ser-318 in response to DNA damage, leading to localization in the nucleus and recruitment to sites of DNA damage. Ubiquitinated.this does not lead to proteasomal degradation. May undergo both 'Lys-48'- and 'Lys-63'-linked polyubiquitination. |
| Function | Regulator of protein degradation that mediates the proteasomal targeting of misfolded, mislocalized or accumulated proteins. Acts by binding polyubiquitin chains of target proteins via its UBA domain and by interacting with subunits of the proteasome via its ubiquitin-like domain. Key regulator of DNA repair that represses homologous recombination repair: in response to DNA damage, recruited to sites of DNA damage following phosphorylation by ATM and acts by binding and removing ubiquitinated MRE11 from damaged chromatin, leading to MRE11 degradation by the proteasome. MRE11 degradation prevents homologous recombination repair, redirecting double-strand break repair toward non-homologous end joining (NHEJ). Specifically recognizes and binds mislocalized transmembrane-containing proteins and targets them to proteasomal degradation. Collaborates with DESI1/POST in the export of ubiquitinated proteins from the nucleus to the cytoplasm. Also plays a role in the regulation of the proteasomal degradation of non-ubiquitinated GJA1. Acts as an adapter protein that recruits UBQLN1 to the autophagy machinery. Mediates the association of UBQLN1 with autophagosomes and the autophagy-related protein LC3 (MAP1LC3A/B/C) and may assist in the maturation of autophagosomes to autolysosomes by mediating autophagosome-lysosome fusion. |
| Protein Name | Ubiquilin-4Ataxin-1 Interacting Ubiquitin-Like ProteinA1upAtaxin-1 Ubiquitin-Like-Interacting Protein A1uConnexin43-Interacting Protein Of 75 KdaCip75 |
| Cellular Localisation | NucleusCytoplasmChromosomeEndoplasmic ReticulumPerinuclear RegionCytoplasmic VesicleAutophagosomeColocalizes With The ProteasomeBoth In Nucleus And CytoplasmExported From The Nucleus Following Interaction With Desi1/PostIn Response To Dna Damage And Phosphorylation At Ser-318 By AtmLocalizes To The Nucleus And Is Recruited To Sites Of Dna Damage |
| Alternative Protein Names | Ubiquilin-4 proteinAtaxin-1 Interacting Ubiquitin-Like Protein proteinA1up proteinAtaxin-1 Ubiquitin-Like-Interacting Protein A1u proteinConnexin43-Interacting Protein Of 75 Kda proteinCip75 proteinUBQLN4 proteinC1orf6 proteinCIP75 proteinUBIN protein |
Information sourced from Uniprot.org
