USP39
Description
The USP39 (ubiquitin specific peptidase 39) is a protein-coding gene located on chromosome 2.
U4/U6.U5 tri-snRNP-associated protein 2 is a protein that in humans is encoded by the USP39 gene.
USP39 (Ubiquitin carboxyl-terminal hydrolase 39) is a deubiquitinating enzyme involved in a wide range of cellular processes, including antiviral responses, epithelial development, DNA repair, and B-cell maturation. It plays a role in pre-mRNA splicing as part of the U4/U6-U5 tri-snRNP complex, a key component of the spliceosome. USP39 specifically regulates immunoglobulin gene rearrangement in a spliceosome-dependent manner, contributing to B-cell development. It also regulates AURKB mRNA levels, influencing cytokinesis and the spindle checkpoint. In response to DNA damage, USP39 deubiquitinates and stabilizes CHK2, regulating apoptosis and the G2/M cell cycle checkpoint. USP39 further plays a crucial role in DNA repair by controlling the recruitment of XRCC4/LIG4 to DNA double-strand breaks for non-homologous end-joining repair. It participates in antiviral activity by affecting type I IFN signaling, stabilizing STAT1 and reducing its 'Lys-6'-linked ubiquitination. USP39 also contributes to non-canonical Wnt signaling during epidermal differentiation and acts as a negative regulator of NF-kappa-B activation by deubiquitinating 'Lys-48'-linked ubiquitination of NFKBIA.
USP39 is also known as 65K, CGI-21, HSPC332, SAD1, SNRNP65.
Associated Diseases
- Griscelli syndrome type 3
- uncombable hair syndrome
- piebald trait-neurologic defects syndrome
- Tietz syndrome
- oculocutaneous albinism type 3
