Aliases for USP18 Gene
External Ids for USP18 Gene
Previous GeneCards Identifiers 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. Diseases associated with USP18 include Band-Like Calcification With Simplified Gyration And Polymicrogyria and Chromosome 22Q11.2 Microduplication Syndrome. Among its related pathways are Ubiquitin-Proteasome Dependent Proteolysis and Immune response IFN alpha/beta signaling pathway. GO annotations related to this gene include cysteine-type endopeptidase activity and thiol-dependent ubiquitinyl hydrolase 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).