FunctionParticipates in the apoptotic response to DNA damage. Isoforms containing the transactivation domain are pro-apoptotic, isoforms lacking the domain are anti-apoptotic and block the function of p53 and transactivating p73 isoforms. May be a tumor suppressor protein.
Tissue specificityExpressed in striatal neurons of patients with Huntington disease (at protein level). Brain, kidney, placenta, colon, heart, liver, spleen, skeletal muscle, prostate, thymus and pancreas. Highly expressed in fetal tissue.
Sequence similaritiesBelongs to the p53 family. Contains 1 SAM (sterile alpha motif) domain.
DomainPossesses an acidic transactivation domain, a central DNA binding domain and a C-terminal oligomerization domain that binds to the ABL tyrosine kinase SH3 domain. The WW-binding motif mediates interaction with WWOX.
Post-translational modificationsIsoform alpha (but not isoform beta) is sumoylated on Lys-627, which potentiates proteasomal degradation but does not affect transcriptional activity. Higher levels of phosphorylation seen in the brain from patients with Huntington disease. Ubiquitinated; leading to its degradation by the proteasome.
Cellular localizationNucleus. Accumulates in the nucleus in response to DNA damage.