Seroatlas · Human Serome Atlas

USP53

Ubiquitin carboxyl-terminal hydrolase 53

Also known as: KIAA1350, UBP53_HUMAN

Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene

Protein identityUniProt · HPA

UniProt accession: Q70EK8
Gene: USP53
Ensembl: ENSG00000145390
Chromosome: 4
Canonical length: 1073 aa
Protein class: Disease related genes, Enzymes, Human disease related genes, Potential drug targets, Predicted intracellular proteins
Subcellular location: Plasma membrane,Cytosol

OverviewNCBI Gene

Enables K63-linked deubiquitinase activity. Predicted to be involved in response to auditory stimulus and sensory perception of sound. Predicted to act upstream of or within action potential; epithelial cell apoptotic process; and outer hair cell apoptotic process. Located in bicellular tight junction. Implicated in progressive familial intrahepatic cholestasis. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

1073 residues, UniProt reviewed canonical sequence.

>Q70EK8|USP53
     1  MAWVKFLRKP GGNLGKVYQP GSMLSLAPTK GLLNEPGQNS CFLNSAVQVL WQLDIFRRSL
    61  RVLTGHVCQG DACIFCALKT IFAQFQHSRE KALPSDNIRH ALAESFKDEQ RFQLGLMDDA
   121  AECFENMLER IHFHIVPSRD ADMCTSKSCI THQKFAMTLY EQCVCRSCGA SSDPLPFTEF
   181  VRYISTTALC NEVERMLERH ERFKPEMFAE LLQAANTTDD YRKCPSNCGQ KIKIRRVLMN
   241  CPEIVTIGLV WDSEHSDLTE AVVRNLATHL YLPGLFYRVT DENAKNSELN LVGMICYTSQ
   301  HYCAFAFHTK SSKWVFFDDA NVKEIGTRWK DVVSKCIRCH FQPLLLFYAN PDGTAVSTED
   361  ALRQVISWSH YKSVAENMGC EKPVIHKSDN LKENGFGDQA KQRENQKFPT DNISSSNRSH
   421  SHTGVGKGPA KLSHIDQREK IKDISRECAL KAIEQKNLLS SQRKDLEKGQ RKDLGRHRDL
   481  VDEDLSHFQS GSPPAPNGFK QHGNPHLYHS QGKGSYKHDR VVPQSRASAQ IISSSKSQIL
   541  APGEKITGKV KSDNGTGYDT DSSQDSRDRG NSCDSSSKSR NRGWKPMRET LNVDSIFSES
   601  EKRQHSPRHK PNISNKPKSS KDPSFSNWPK ENPKQKGLMT IYEDEMKQEI GSRSSLESNG
   661  KGAEKNKGLV EGKVHGDNWQ MQRTESGYES SDHISNGSTN LDSPVIDGNG TVMDISGVKE
   721  TVCFSDQITT SNLNKERGDC TSLQSQHHLE GFRKELRNLE AGYKSHEFHP ESHLQIKNHL
   781  IKRSHVHEDN GKLFPSSSLQ IPKDHNAREH IHQSDEQKLE KPNECKFSEW LNIENSERTG
   841  LPFHVDNSAS GKRVNSNEPS SLWSSHLRTV GLKPETAPLI QQQNIMDQCY FENSLSTECI
   901  IRSASRSDGC QMPKLFCQNL PPPLPPKKYA ITSVPQSEKS ESTPDVKLTE VFKATSHLPK
   961  HSLSTASEPS LEVSTHMNDE RHKETFQVRE CFGNTPNCPS SSSTNDFQAN SGAIDAFCQP
  1021  ELDSISTCPN ETVSLTTYFS VDSCMTDTYR LKYHQRPKLS FPESSGFCNN SLS

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against USP53 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability: Cell surface
Secreted: No
Transmembrane segments: 0
Mean surface accessibility (rSASA): 0.55
Highest tissue expression: 41 nTPM

Expression across tissuesHPA

Tissue

tongue: 41 nTPM
adipose tissue: 31 nTPM
fallopian tube: 24 nTPM
breast: 21 nTPM
heart muscle: 19 nTPM
pancreas: 19 nTPM

Single-cell type

endometrial glandular cells: 2,703 nCPM
endometrial luminal cells: 992 nCPM
thymic myoid cells: 658 nCPM
microglia: 602 nCPM
colonocytes: 552 nCPM
ocular epithelial cells: 546 nCPM

Immune cell

naive CD4 T-cell: 1.8 nTPM
basophil: 1.1 nTPM
memory CD4 T-cell: 0.8 nTPM
memory CD8 T-cell: 0.8 nTPM
naive CD8 T-cell: 0.8 nTPM
T-reg: 0.8 nTPM

Brain region

choroid plexus: 29 nTPM
medulla oblongata: 23 nTPM
thalamus: 22 nTPM
midbrain: 20 nTPM
hypothalamus: 18 nTPM
pons: 17 nTPM

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about USP53.

Disease | AllUniProt

Conditions USP53 is implicated in, by any mechanism.

Cholestasis, progressive familial intrahepatic, 7, with or without hearing loss (PFIC7) MIM:619658

Disease | GeneticClinVar

41 pathogenic / likely-pathogenic of 271 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

Cholestasis, progressive familial intrahepatic, 7, with or without hearing loss
Cholestasis
USP53-related disorder
Cholestasis, progressive familial intrahepatic, (PFIC4-like)
See cases

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance): 0.78
gnomAD pLI: 0
gnomAD missense Z: 0.98
DepMap mean gene effect: 0.04
DepMap dependency class: none

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 3% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

Molecular functions

Cellular components

Protein domainsUniProt · Pfam · InterPro

KeywordsUniProt

InteractionsUniProt · HPA

Protein binding partners of USP53 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

Antibody and autoantibody relevanceSeroatlas analysis

Seroatlas reads USP53 as an antibody target. Whether an autoantibody or antibody against USP53 could matter depends on whether native USP53 is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.

USP53 is annotated at the cell surface, where native USP53 is exposed to circulating antibodies and is a prime autoantibody target that could block, deplete, or overstimulate it.

Annotation status

The present source text does not explicitly label USP53 as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.

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