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Protein

Ubiquitin carboxyl-terminal hydrolase 27

Gene

USP27X

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Deubiquitinase that can reduce the levels of BCL2L11/BIM ubiquitination and stabilize BCL2L11 in response to the RAF-MAPK-degradation signal. By acting on BCL2L11 levels, may counteract the anti-apoptotic effects of MAPK activity.By similarity

Catalytic activityi

Thiol-dependent hydrolysis of ester, thioester, amide, peptide and isopeptide bonds formed by the C-terminal Gly of ubiquitin (a 76-residue protein attached to proteins as an intracellular targeting signal).

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei87NucleophilePROSITE-ProRule annotation1
Active sitei380Proton acceptorPROSITE-ProRule annotation1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionHydrolase, Protease, Thiol protease
Biological processUbl conjugation pathway

Names & Taxonomyi

Protein namesi
Recommended name:
Ubiquitin carboxyl-terminal hydrolase 27 (EC:3.4.19.12)
Alternative name(s):
Deubiquitinating enzyme 27
Ubiquitin carboxyl-terminal hydrolase 22-like
Ubiquitin thioesterase 27
Ubiquitin-specific-processing protease 27
X-linked ubiquitin carboxyl-terminal hydrolase 27
Gene namesi
Name:USP27X
Synonyms:USP22L, USP27
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome X

Organism-specific databases

EuPathDBiHostDB:ENSG00000273820.1.
HGNCiHGNC:13486. USP27X.

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm, Nucleus

Pathology & Biotechi

Involvement in diseasei

Mental retardation, X-linked 105 (MRX105)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA form of mental retardation, a disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptive behavior and manifested during the developmental period. Intellectual deficiency is the only primary symptom of non-syndromic X-linked mental retardation, while syndromic mental retardation presents with associated physical, neurological and/or psychiatric manifestations.
See also OMIM:300984
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_077830381Y → H in MRX105. 1 Publication1

Keywords - Diseasei

Disease mutation, Mental retardation

Organism-specific databases

MalaCardsiUSP27X.
MIMi300984. phenotype.
OpenTargetsiENSG00000273820.
PharmGKBiPA134993614.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00003063341 – 438Ubiquitin carboxyl-terminal hydrolase 27Add BLAST438

Proteomic databases

EPDiA6NNY8.
MaxQBiA6NNY8.
PRIDEiA6NNY8.

PTM databases

iPTMnetiA6NNY8.
PhosphoSitePlusiA6NNY8.

Expressioni

Gene expression databases

BgeeiENSG00000273820.
CleanExiHS_USP27X.
GenevisibleiA6NNY8. HS.

Organism-specific databases

HPAiHPA077264.

Interactioni

Subunit structurei

Interacts with phosphorylated BCL2L11 isoform BIMEL; this interaction leads to BCL2L11 deubiquitination and stabilization.By similarity

Protein-protein interaction databases

BioGridi133302. 7 interactors.
IntActiA6NNY8. 1 interactor.

Structurei

3D structure databases

ProteinModelPortaliA6NNY8.
SMRiA6NNY8.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini78 – 421USPAdd BLAST344

Sequence similaritiesi

Belongs to the peptidase C19 family.Curated

Phylogenomic databases

GeneTreeiENSGT00890000139343.
HOVERGENiHBG058014.
InParanoidiA6NNY8.
KOiK11366.
OMAiTEKHIHE.
OrthoDBiEOG091G09GI.
PhylomeDBiA6NNY8.

Family and domain databases

InterProiView protein in InterPro
IPR001394. Peptidase_C19_UCH.
IPR018200. USP_CS.
IPR028889. USP_dom.
PfamiView protein in Pfam
PF00443. UCH. 1 hit.
PROSITEiView protein in PROSITE
PS00972. USP_1. 1 hit.
PS00973. USP_2. 1 hit.
PS50235. USP_3. 1 hit.

Sequencei

Sequence statusi: Complete.

A6NNY8-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MCKDYVYDKD IEQIAKEEQG EALKLQASTS TEVSHQQCSV PGLGEKFPTW
60 70 80 90 100
ETTKPELELL GHNPRRRRIT SSFTIGLRGL INLGNTCFMN CIVQALTHTP
110 120 130 140 150
ILRDFFLSDR HRCEMPSPEL CLVCEMSSLF RELYSGNPSP HVPYKLLHLV
160 170 180 190 200
WIHARHLAGY RQQDAHEFLI AALDVLHRHC KGDDVGKAAN NPNHCNCIID
210 220 230 240 250
QIFTGGLQSD VTCQACHGVS TTIDPCWDIS LDLPGSCTSF WPMSPGRESS
260 270 280 290 300
VNGESHIPGI TTLTDCLRRF TRPEHLGSSA KIKCGSCQSY QESTKQLTMN
310 320 330 340 350
KLPVVACFHF KRFEHSAKQR RKITTYISFP LELDMTPFMA SSKESRMNGQ
360 370 380 390 400
LQLPTNSGNN ENKYSLFAVV NHQGTLESGH YTSFIRHHKD QWFKCDDAVI
410 420 430
TKASIKDVLD SEGYLLFYHK QVLEHESEKV KEMNTQAY
Length:438
Mass (Da):49,630
Last modified:March 24, 2009 - v3
Checksum:iB6BFD18C62B3774C
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti32E → D in DR004246 (Ref. 2) Curated1
Sequence conflicti45E → D in DR004246 (Ref. 2) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_077830381Y → H in MRX105. 1 Publication1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF238380 Genomic DNA. No translation available.
DR004246 mRNA. No translation available.
CCDSiCCDS65260.1.
RefSeqiNP_001138545.1. NM_001145073.2.
UniGeneiHs.143587.

Genome annotation databases

EnsembliENST00000621775; ENSP00000483631; ENSG00000273820.
GeneIDi389856.
KEGGihsa:389856.
UCSCiuc033edm.2. human.

Similar proteinsi

Entry informationi

Entry nameiUBP27_HUMAN
AccessioniPrimary (citable) accession number: A6NNY8
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 2, 2007
Last sequence update: March 24, 2009
Last modified: October 25, 2017
This is version 87 of the entry and version 3 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Caution

Although strongly related to USP22, which deubiquitinates histones, lacks the N-terminal UBP-type zinc finger, suggesting it does not have the ability to deubiquitinate histones.Curated

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome X
    Human chromosome X: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. Peptidase families
    Classification of peptidase families and list of entries
  6. SIMILARITY comments
    Index of protein domains and families