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Q9BXU7 (UBP26_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 104. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (6) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Ubiquitin carboxyl-terminal hydrolase 26

EC=3.4.19.12
Alternative name(s):
Deubiquitinating enzyme 26
Ubiquitin thioesterase 26
Ubiquitin-specific-processing protease 26
Gene names
Name:USP26
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length913 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Involved in the ubiquitin-dependent proteolytic pathway in conjunction with the 26S proteasome By similarity. Deubiquitinates the androgen receptor and regulates the androgen receptor signaling pathway. Ref.4

Catalytic activity

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).

Subunit structure

Interacts with AR. Ref.4

Subcellular location

Nucleus Ref.4.

Sequence similarities

Belongs to the peptidase C19 family.

Contains 1 USP domain.

Ontologies

Keywords
   Biological processUbl conjugation pathway
   Cellular componentNucleus
   Coding sequence diversityPolymorphism
   Molecular functionHydrolase
Protease
Thiol protease
   Technical termComplete proteome
Reference proteome
Gene Ontology (GO)
   Biological_processprotein deubiquitination

Inferred from direct assay Ref.4. Source: UniProtKB

ubiquitin-dependent protein catabolic process

Inferred from electronic annotation. Source: InterPro

   Cellular_componentnucleus

Inferred from direct assay Ref.4. Source: UniProtKB

   Molecular_functioncysteine-type peptidase activity

Inferred from electronic annotation. Source: UniProtKB-KW

Complete GO annotation...

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 913913Ubiquitin carboxyl-terminal hydrolase 26
PRO_0000080655

Regions

Domain295 – 886592USP

Sites

Active site3041Nucleophile By similarity
Active site8411Proton acceptor By similarity

Natural variations

Natural variant1231T → TT. Ref.7
VAR_063413
Natural variant1651L → S. Ref.7
Corresponds to variant rs61741870 [ dbSNP | Ensembl ].
VAR_063414
Natural variant3641L → F. Ref.5 Ref.6 Ref.7
Corresponds to variant rs35397110 [ dbSNP | Ensembl ].
VAR_063415
Natural variant4751H → Y. Ref.5 Ref.7
Corresponds to variant rs41299088 [ dbSNP | Ensembl ].
VAR_063416
Natural variant5171L → F. Ref.6
VAR_063417
Natural variant5791M → I. Ref.7
Corresponds to variant rs138385391 [ dbSNP | Ensembl ].
VAR_063418

Experimental info

Mutagenesis3041C → S: Results in increased AR signaling. Ref.4

Sequences

Sequence LengthMass (Da)Tools
Q9BXU7 [UniParc].

Last modified June 1, 2001. Version 1.
Checksum: BB70B09CDCBA3C48

FASTA913104,047
        10         20         30         40         50         60 
MAALFLRGFV QIGNCKTGIS KSKEAFIEAV ERKKKDRLVL YFKSGKYSTF RLSDNIQNVV 

        70         80         90        100        110        120 
LKSYRGNQNH LHLTLQNNNG LFIEGLSSTD AEQLKIFLDR VHQNEVQPPV RPGKGGSVFS 

       130        140        150        160        170        180 
STTQKEINKT SFHKVDEKSS SKSFEIAKGS GTGVLQRMPL LTSKLTLTCG ELSENQHKKR 

       190        200        210        220        230        240 
KRMLSSSSEM NEEFLKENNS VEYKKSKADC SRCVSYNREK QLKLKELEEN KKLECESSCI 

       250        260        270        280        290        300 
MNATGNPYLD DIGLLQALTE KMVLVFLLQQ GYSDGYTKWD KLKLFFELFP EKICHGLPNL 

       310        320        330        340        350        360 
GNTCYMNAVL QSLLSIPSFA DDLLNQSFPW GKIPLNALTM CLARLLFFKD TYNIEIKEML 

       370        380        390        400        410        420 
LLNLKKAISA AAEIFHGNAQ NDAHEFLAHC LDQLKDNMEK LNTIWKPKSE FGEDNFPKQV 

       430        440        450        460        470        480 
FADDPDTSGF SCPVITNFEL ELLHSIACKA CGQVILKTEL NNYLSINLPQ RIKAHPSSIQ 

       490        500        510        520        530        540 
STFDLFFGAE ELEYKCAKCE HKTSVGVHSF SRLPRILIVH LKRYSLNEFC ALKKNDQEVI 

       550        560        570        580        590        600 
ISKYLKVSSH CNEGTRPPLP LSEDGEITDF QLLKVIRKMT SGNISVSWPA TKESKDILAP 

       610        620        630        640        650        660 
HIGSDKESEQ KKGQTVFKGA SRRQQQKYLG KNSKPNELES VYSGDRAFIE KEPLAHLMTY 

       670        680        690        700        710        720 
LEDTSLCQFH KAGGKPASSP GTPLSKVDFQ TVPENPKRKK YVKTSKFVAF DRIINPTKDL 

       730        740        750        760        770        780 
YEDKNIRIPE RFQKVSEQTQ QCDGMRICEQ APQQALPQSF PKPGTQGHTK NLLRPTKLNL 

       790        800        810        820        830        840 
QKSNRNSLLA LGSNKNPRNK DILDKIKSKA KETKRNDDKG DHTYRLISVV SHLGKTLKSG 

       850        860        870        880        890        900 
HYICDAYDFE KQIWFTYDDM RVLGIQEAQM QEDRRCTGYI FFYMHNEIFE EMLKREENAQ 

       910 
LNSKEVEETL QKE 

« Hide

References

« Hide 'large scale' references
[1]"An abundance of X-linked genes expressed in spermatogonia."
Wang P.J., McCarrey J.R., Yang F., Page D.C.
Nat. Genet. 27:422-426(2001) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA].
Tissue: Testis.
[2]"The DNA sequence of the human X chromosome."
Ross M.T., Grafham D.V., Coffey A.J., Scherer S., McLay K., Muzny D., Platzer M., Howell G.R., Burrows C., Bird C.P., Frankish A., Lovell F.L., Howe K.L., Ashurst J.L., Fulton R.S., Sudbrak R., Wen G., Jones M.C. expand/collapse author list , Hurles M.E., Andrews T.D., Scott C.E., Searle S., Ramser J., Whittaker A., Deadman R., Carter N.P., Hunt S.E., Chen R., Cree A., Gunaratne P., Havlak P., Hodgson A., Metzker M.L., Richards S., Scott G., Steffen D., Sodergren E., Wheeler D.A., Worley K.C., Ainscough R., Ambrose K.D., Ansari-Lari M.A., Aradhya S., Ashwell R.I., Babbage A.K., Bagguley C.L., Ballabio A., Banerjee R., Barker G.E., Barlow K.F., Barrett I.P., Bates K.N., Beare D.M., Beasley H., Beasley O., Beck A., Bethel G., Blechschmidt K., Brady N., Bray-Allen S., Bridgeman A.M., Brown A.J., Brown M.J., Bonnin D., Bruford E.A., Buhay C., Burch P., Burford D., Burgess J., Burrill W., Burton J., Bye J.M., Carder C., Carrel L., Chako J., Chapman J.C., Chavez D., Chen E., Chen G., Chen Y., Chen Z., Chinault C., Ciccodicola A., Clark S.Y., Clarke G., Clee C.M., Clegg S., Clerc-Blankenburg K., Clifford K., Cobley V., Cole C.G., Conquer J.S., Corby N., Connor R.E., David R., Davies J., Davis C., Davis J., Delgado O., Deshazo D., Dhami P., Ding Y., Dinh H., Dodsworth S., Draper H., Dugan-Rocha S., Dunham A., Dunn M., Durbin K.J., Dutta I., Eades T., Ellwood M., Emery-Cohen A., Errington H., Evans K.L., Faulkner L., Francis F., Frankland J., Fraser A.E., Galgoczy P., Gilbert J., Gill R., Gloeckner G., Gregory S.G., Gribble S., Griffiths C., Grocock R., Gu Y., Gwilliam R., Hamilton C., Hart E.A., Hawes A., Heath P.D., Heitmann K., Hennig S., Hernandez J., Hinzmann B., Ho S., Hoffs M., Howden P.J., Huckle E.J., Hume J., Hunt P.J., Hunt A.R., Isherwood J., Jacob L., Johnson D., Jones S., de Jong P.J., Joseph S.S., Keenan S., Kelly S., Kershaw J.K., Khan Z., Kioschis P., Klages S., Knights A.J., Kosiura A., Kovar-Smith C., Laird G.K., Langford C., Lawlor S., Leversha M., Lewis L., Liu W., Lloyd C., Lloyd D.M., Loulseged H., Loveland J.E., Lovell J.D., Lozado R., Lu J., Lyne R., Ma J., Maheshwari M., Matthews L.H., McDowall J., McLaren S., McMurray A., Meidl P., Meitinger T., Milne S., Miner G., Mistry S.L., Morgan M., Morris S., Mueller I., Mullikin J.C., Nguyen N., Nordsiek G., Nyakatura G., O'dell C.N., Okwuonu G., Palmer S., Pandian R., Parker D., Parrish J., Pasternak S., Patel D., Pearce A.V., Pearson D.M., Pelan S.E., Perez L., Porter K.M., Ramsey Y., Reichwald K., Rhodes S., Ridler K.A., Schlessinger D., Schueler M.G., Sehra H.K., Shaw-Smith C., Shen H., Sheridan E.M., Shownkeen R., Skuce C.D., Smith M.L., Sotheran E.C., Steingruber H.E., Steward C.A., Storey R., Swann R.M., Swarbreck D., Tabor P.E., Taudien S., Taylor T., Teague B., Thomas K., Thorpe A., Timms K., Tracey A., Trevanion S., Tromans A.C., d'Urso M., Verduzco D., Villasana D., Waldron L., Wall M., Wang Q., Warren J., Warry G.L., Wei X., West A., Whitehead S.L., Whiteley M.N., Wilkinson J.E., Willey D.L., Williams G., Williams L., Williamson A., Williamson H., Wilming L., Woodmansey R.L., Wray P.W., Yen J., Zhang J., Zhou J., Zoghbi H., Zorilla S., Buck D., Reinhardt R., Poustka A., Rosenthal A., Lehrach H., Meindl A., Minx P.J., Hillier L.W., Willard H.F., Wilson R.K., Waterston R.H., Rice C.M., Vaudin M., Coulson A., Nelson D.L., Weinstock G., Sulston J.E., Durbin R.M., Hubbard T., Gibbs R.A., Beck S., Rogers J., Bentley D.R.
Nature 434:325-337(2005) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[3]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
[4]"The deubiquitinating enzyme USP26 is a regulator of androgen receptor signaling."
Dirac A.M., Bernards R.
Mol. Cancer Res. 8:844-854(2010) [PubMed] [Europe PMC] [Abstract]
Cited for: FUNCTION, SUBCELLULAR LOCATION, MUTAGENESIS OF CYS-304, INTERACTION WITH AR.
[5]"Novel mutations in testis-specific ubiquitin protease 26 gene may cause male infertility and hypogonadism."
Paduch D.A., Mielnik A., Schlegel P.N.
Reprod. BioMed. Online 10:747-754(2005) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANTS PHE-364 AND TYR-475.
[6]"Sequence analysis of the X-linked USP26 gene in severe male factor infertility patients and fertile controls."
Christensen G.L., Griffin J., Carrell D.T.
Fertil. Steril. 90:851-852(2008) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANTS PHE-364 AND PHE-517.
[7]"USP26 gene variations in fertile and infertile men."
Ribarski I., Lehavi O., Yogev L., Hauser R., Bar-Shira Maymon B., Botchan A., Paz G., Yavetz H., Kleiman S.E.
Hum. Reprod. 24:477-484(2009) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANTS THR-123 INS; SER-165; PHE-364; TYR-475 AND ILE-579.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
AF285593 mRNA. Translation: AAK31972.1.
Z81365 Genomic DNA. Translation: CAX30813.1.
BC069073 mRNA. Translation: AAH69073.1.
BC101190 mRNA. Translation: AAI01191.1.
BC101191 mRNA. Translation: AAI01192.1.
RefSeqNP_114113.1. NM_031907.1.
UniGeneHs.333137.

3D structure databases

ProteinModelPortalQ9BXU7.
SMRQ9BXU7. Positions 16-106, 296-525.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid123764. 4 interactions.
IntActQ9BXU7. 2 interactions.
STRING9606.ENSP00000359869.

Protein family/group databases

MEROPSC19.046.

PTM databases

PhosphoSiteQ9BXU7.

Polymorphism databases

DMDM18202739.

Proteomic databases

PaxDbQ9BXU7.
PRIDEQ9BXU7.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000370832; ENSP00000359869; ENSG00000134588.
ENST00000406273; ENSP00000384360; ENSG00000134588.
ENST00000511190; ENSP00000423390; ENSG00000134588.
GeneID83844.
KEGGhsa:83844.
UCSCuc010nrm.1. human.

Organism-specific databases

CTD83844.
GeneCardsGC0XM132158.
HGNCHGNC:13485. USP26.
MIM300309. gene.
neXtProtNX_Q9BXU7.
PharmGKBPA37782.
GenAtlasSearch...

Phylogenomic databases

eggNOGCOG5533.
HOGENOMHOG000060197.
HOVERGENHBG055893.
InParanoidQ9BXU7.
KOK11850.
OMADAYDFER.
OrthoDBEOG7HMS09.
PhylomeDBQ9BXU7.
TreeFamTF323032.

Gene expression databases

BgeeQ9BXU7.
CleanExHS_USP26.
GenevestigatorQ9BXU7.

Family and domain databases

InterProIPR018200. Pept_C19ubi-hydrolase_C_CS.
IPR001394. Peptidase_C19_UCH.
IPR028889. UCH/PAN2.
[Graphical view]
PfamPF00443. UCH. 1 hit.
[Graphical view]
PROSITEPS00972. USP_1. 1 hit.
PS00973. USP_2. 1 hit.
PS50235. USP_3. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

GenomeRNAi83844.
NextBio72811.
PROQ9BXU7.
SOURCESearch...

Entry information

Entry nameUBP26_HUMAN
AccessionPrimary (citable) accession number: Q9BXU7
Secondary accession number(s): B9WRT6, Q5H9H4
Entry history
Integrated into UniProtKB/Swiss-Prot: September 26, 2001
Last sequence update: June 1, 2001
Last modified: April 16, 2014
This is version 104 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (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.

Relevant documents

SIMILARITY comments

Index of protein domains and families

Peptidase families

Classification of peptidase families and list of entries

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human chromosome X

Human chromosome X: entries, gene names and cross-references to MIM