HELZ2

Multifunctional nuclear protein involved in RNA surveillance, nuclear receptor-mediated transcriptional regulation, and the restriction of mobile genetic elements as part of the innate immune response. Degrades highly structured RNAs through its concerted ATP-dependent RNA helicase and 3' to 5' exoribonuclease activities. Shows a strong preference for pyrimidine over purine residues for its nuclease activity. Acts as a transcriptional coactivator for a number of nuclear receptors including PPARA, PPARG, THRA, THRB and RXRA. In addition, regulates lipid metabolism independently of PPARs by controlling the stability of Apob mRNA, which encodes a protein essential for lipoprotein assembly and secretion. Also contributes to adipocyte differentiation through interactions with the splicing factor proline- and glutamine-rich protein SFPQ and THRAP3..

Function from UniProtKB (Q9BYK8), read 2026-09-23. CC BY 4.0.