Ubiquitin Specific Peptidase 54 (USP54)

The ubiquitin (Ub) pathway involves three sequential enzymatic steps that facilitate the conjugation of Ub and Ub-like molecules to specific protein substrates. Through the use of a wide range of enzymes that can add or remove ubiquitin, the Ub pathway controls many intracellular processes such as signal transduction, transcriptional activation and cell cycle progression. USP54 (ubiquitin specific peptidase 54) is a 1,684 amino acid protein that belongs to the peptidase C19 family. Weakly expressed in a few tissues, USP54 contains an inactive ubiquitin carboxyl-terminal hydrolase domain, a katanin-like ATPase KATNAL1 motif and an uncharacterized peptide stretch containing a VPS9 domain. USP54 is encoded by a gene located on human chromosome 10, which houses over 1,200 genes and comprises nearly 4.5% of the human genome.