USP20

Description

The USP20 (ubiquitin specific peptidase 20) is a protein-coding gene located on chromosome 9.

Ubiquitin carboxyl-terminal hydrolase 20 is an enzyme that in humans is encoded by the USP20 gene. Ubiquitin-specific protease 20 (USP20), also known as ubiquitin-binding protein 20 and VHL protein-interacting deubiquitinating enzyme 2 (VDU2), is a cysteine protease deubiquitinating enzyme (DUB). The catalytic site of USP20, like other DUBs, contains conserved cysteine and histidine residues that catalyse the proteolysis of an isopeptide bond between a lysine residue of a target protein and a glycine residue of a ubiquitin molecule. USP20 is known to deubiquitinate a number of proteins including thyronine deiodinase type 2 (D2), Hypoxia-inducible factor 1α (HIF1α), and β2 adrenergic receptor (β2AR).

== Gene == The USP20 gene is located on chromosome 9 at the locus 9q34.11.

== Structure == USP20 is a 914-amino acid protein that shows 59% homology with another DUB, USP33. It contains 4 known domains, an N-terminal Zf UBP domain, a catalytic domain containing conserved histidine and cysteine residues, and two C-terminal DUSP domains.

== Function == DUBs are categorised into 5 main groups, ubiquitin-specific proteases (USP), ubiquitin c-terminal hydrolases (UCH), ovarian tumour proteases (OTU), Machado-Joseph disease proteases (MJD), and JAB1/MPN/MOV34 proteases (JAMM/MPN+). The first four groups are cysteine proteases, whereas the last group are Zn metalloproteases. USP20 belongs to the USP group and, like most DUBs, catalyse the breakage of an isopeptide bond between a lysine residue of the target protein and the terminal glycine residue of a ubiquitin protein.

USP20 is a deubiquitinating enzyme involved in a variety of cellular processes, including autophagy, antiviral responses, and membrane protein biogenesis. It plays a role in regulating the stability of proteins like ULK1, STING1, and SQSTM1, which are critical for autophagy and antiviral signaling. USP20 also regulates the activity of G-protein coupled receptors (GPCRs) by deubiquitinating the beta-2 adrenergic receptor (ADRB2). It interacts with various proteins including VHL, CCP110, DIO2, HIF1A, ADRB2, and USP18, which suggests its involvement in complex signaling pathways. Additionally, USP20 works with USP33 to rescue improperly ubiquitinated membrane proteins in the endoplasmic reticulum.

USP20 is also known as LSFR3A, VDU2, hVDU2.

Associated Diseases

WES Whole Exome Sequencing