Aliases for USP18 Gene
External Ids for USP18 Gene
The protein encoded by this gene belongs to the ubiquitin-specific proteases (UBP) family of enzymes that cleave ubiquitin from ubiquitinated protein substrates. It is highly expressed in liver and thymus, and is localized to the nucleus. This protein efficiently cleaves only ISG15 (a ubiquitin-like protein) fusions, and deletion of this gene in mice results in a massive increase of ISG15 conjugates in tissues, indicating that this protein is a major ISG15-specific protease. Mice lacking this gene are also hypersensitive to interferon, suggesting a function of this protein in downregulating interferon responses, independent of its isopeptidase activity towards ISG15. [provided by RefSeq, Sep 2011]
GeneCards Summary for USP18 Gene
USP18 (Ubiquitin Specific Peptidase 18) is a Protein Coding gene. Among its related pathways are Interferon Signaling and Interferon Signaling. GO annotations related to this gene include ubiquitin thiolesterase activity and ubiquitin-specific protease activity. An important paralog of this gene is USP41.
UniProtKB/Swiss-Prot for USP18 Gene
Can efficiently cleave only ISG15 fusions including native ISG15 conjugates linked via isopeptide bonds. Necessary to maintain a critical cellular balance of ISG15-conjugated proteins in both healthy and stressed organisms
Isoform 2: Has enzymatic activity similar to isoform 1 and interferes with type I interferon signaling. Major deISGylation enzyme for nuclear proteins (PubMed:22170061).