Proteins/UNIPROT:A6NNY8

USP27X

ProteinUNIPROT:A6NNY8

Deubiquitinase involved in innate antiviral immunity by mediating deubiquitination of CGAS and RIGI. Negatively regulates RIGI by mediating 'Lys-63'-linked deubiquitination of RIGI, inhibiting type I interferon signaling. Also regulates 'Lys-63'-linked ubiquitination level of MDA5/IFIH1. Acts as a positive regulator of the cGAS-STING pathway by catalyzing 'Lys-48'-linked deubiquitination of CGAS, thereby promoting its stabilization. Can reduce the levels of BCL2L11/BIM ubiquitination and stabilize BCL2L11 in response to the RAF-MAPK-degradation signal (By similarity). By acting on BCL2L11 levels, may counteract the anti-apoptotic effects of MAPK activity (By similarity)..

Function from UniProtKB (A6NNY8), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

3 rows

intellectual disability, X-linked 105 has a germline basis in USP27XDiseaseGenCC HGNC:13486|MONDO:0010510Inferred only
non-syndromic X-linked intellectual disability has a germline basis in USP27XDiseaseGenCC HGNC:13486|MONDO:0019181Inferred only
X-linked complex neurodevelopmental disorder has a germline basis in USP27XDiseaseGenCC HGNC:13486|MONDO:0100148Inferred only

Statements

11 statements

11 statements · 2 reference databases · Subject: USP27X · Sources: gene ontology, Hetionet

Order

USP27X covaries with BAZ1BProteinHetionet hetionet:covaries:389856-9031Inferred only
USP27X covaries with INTUProteinHetionet hetionet:covaries:27152-389856Inferred only
MAPT covaries with USP27XProteinHetionet hetionet:covaries:389856-4137Inferred only
PLP1 covaries with USP27XProteinHetionet hetionet:covaries:389856-5354Inferred only
USP27X covaries with PTP4A3ProteinHetionet hetionet:covaries:11156-389856Inferred only
USP27X covaries with TNFSF15ProteinHetionet hetionet:covaries:389856-9966Inferred only
TNRC6B covaries with USP27XProteinHetionet hetionet:covaries:23112-389856Inferred only
VWA5B1 covaries with USP27XProteinHetionet hetionet:covaries:127731-389856Inferred only
UBC interacts with USP27XProteinHetionet hetionet:interacts:389856-7316Inferred only
USP27X regulates positive regulation of apoptotic processBiological processgene ontology A6NNY8|GO:0043065Inferred only
USP27X regulates positive regulation of type I interferon-mediated signaling pathwayBiological processgene ontology A6NNY8|GO:0060340Inferred only

Reactions

1 reaction

water+MAM03104cproton+a thiol+MAM03105c
Catalysed by USP2 or USP28 or USP36 or USP13 or OTUD5 or USP33 or CYLD or USP40 or USP48 or USP14 or USP10 or USP31 and 43 more alternatives
Reaction pageLeft to rightBalance undeterminable

Other names

7 names

Resolves to: USP27X

Also called: Deubiquitinating enzyme 27Ubiquitin carboxyl-terminal hydrolase 22-likeUbiquitin thioesterase 27Ubiquitin-specific-processing protease 27USP22LUSP27X-linked ubiquitin carboxyl-terminal hydrolase 27

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