Skip Header

You are using a version of Internet Explorer that may not display all features of this website. Please upgrade to a modern browser.
Contribute Send feedback
Read comments (?) or add your own

Q9UKY1 (ZHX1_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 122. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (4) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Interactions·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Zinc fingers and homeoboxes protein 1
Gene names
Name:ZHX1
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length873 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Acts as a transcriptional repressor. Increases DNMT3B-mediated repressive transcriptional activity when DNMT3B is tethered to DNA. May link molecule between DNMT3B and other co-repressor proteins. Ref.5

Subunit structure

Forms homodimers. Heterodimer (via HD1 domain) with ZHX2 (via HD1 domain). Also forms a heterodimer with ZHX3 which is a prerequisite for repressor activity. Interacts with ATF7IP and NFYA. Interacts (via homeobox domains) with DNMT3B (via PWWP domain). Ref.1 Ref.4 Ref.5 Ref.6 Ref.7 Ref.10

Subcellular location

Nucleus. Note: Colocalized in the nucleus with DNMT3B. Ref.5 Ref.8

Tissue specificity

Ubiquitously expressed. Expressed in podocytes. Ref.1 Ref.8

Sequence similarities

Belongs to the ZHX family.

Contains 2 C2H2-type zinc fingers.

Contains 5 homeobox DNA-binding domains.

Binary interactions

With

Entry

#Exp.

IntAct

Notes

ATXN1P542535EBI-347767,EBI-930964
DNMT3BQ9UBC3-14EBI-347767,EBI-6083193

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]
Isoform 1 (identifier: Q9UKY1-1)

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.
Isoform 2 (identifier: Q96EF9-1)

Also known as: ZHX1-C8orf76;

The sequence of this isoform can be found in the external entry Q96EF9.
Isoforms of the same protein are often annotated in two different entries if their sequences differ significantly.
Note: Based on a readthrough transcript which may produce a ZHX1-C8orf76 fusion protein. No experimental confirmation available.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 873873Zinc fingers and homeoboxes protein 1
PRO_0000049388

Regions

Zinc finger70 – 9324C2H2-type 1
Zinc finger102 – 12524C2H2-type 2
DNA binding284 – 34663Homeobox 1
DNA binding464 – 52663Homeobox 2
DNA binding569 – 63062Homeobox 3
DNA binding660 – 72263Homeobox 4
DNA binding777 – 83256Homeobox 5
Region272 – 564293Required for interaction with NFYA Ref.4
Region272 – 432161Required for dimerization Ref.5
Region734 – 76835Required for nuclear localization Ref.5
Region831 – 87343Required for repressor activity Ref.5

Amino acid modifications

Modified residue451Phosphoserine Ref.11
Modified residue471Phosphoserine Ref.11 Ref.13
Modified residue481Phosphoserine Ref.11 Ref.13
Modified residue6481Phosphoserine Ref.13

Experimental info

Sequence conflict2631V → M in AAH40481. Ref.3
Sequence conflict7921H → R in AAH40481. Ref.3

Secondary structure

............................................. 873
Helix Strand Turn

Details...

Sequences

Sequence LengthMass (Da)Tools
Isoform 1 [UniParc].

Last modified May 1, 2000. Version 1.
Checksum: 66CF1CEC5EF824E5

FASTA87398,098
        10         20         30         40         50         60 
MASRRKSTTP CMVLASEQDP DLELISDLDE GPPVLTPVEN TRAESISSDE EVHESVDSDN 

        70         80         90        100        110        120 
QQNKKVEGGY ECKYCTFQTP DLNMFTFHVD SEHPNVVLNS SYVCVECNFL TKRYDALSEH 

       130        140        150        160        170        180 
NLKYHPGEEN FKLTMVKRNN QTIFEQTIND LTFDGSFVKE ENAEQAESTE VSSSGISISK 

       190        200        210        220        230        240 
TPIMKMMKNK VENKRIAVHH NSVEDVPEEK ENEIKPDREE IVENPSSSAS ESNTSTSIVN 

       250        260        270        280        290        300 
RIHPSTASTV VTPAAVLPGL AQVITAVSAQ QNSNLIPKVL IPVNSIPTYN AALDNNPLLL 

       310        320        330        340        350        360 
NTYNKFPYPT MSEITVLSAQ AKYTEEQIKI WFSAQRLKHG VSWTPEEVEE ARRKQFNGTV 

       370        380        390        400        410        420 
HTVPQTITVI PTHISTGSNG LPSILQTCQI VGQPGLVLTQ VAGTNTLPVT APIALTVAGV 

       430        440        450        460        470        480 
PSQNNIQKSQ VPAAQPTAET KPATAAVPTS QSVKHETALV NPDSFGIRAK KTKEQLAELK 

       490        500        510        520        530        540 
VSYLKNQFPH DSEIIRLMKI TGLTKGEIKK WFSDTRYNQR NSKSNQCLHL NNDSSTTIII 

       550        560        570        580        590        600 
DSSDETTESP TVGTAQPKQS WNPFPDFTPQ KFKEKTAEQL RVLQASFLNS SVLTDEELNR 

       610        620        630        640        650        660 
LRAQTKLTRR EIDAWFTEKK KSKALKEEKM EIDESNAGSS KEEAGETSPA DESGAPKSGS 

       670        680        690        700        710        720 
TGKICKKTPE QLHMLKSAFV RTQWPSPEEY DKLAKESGLA RTDIVSWFGD TRYAWKNGNL 

       730        740        750        760        770        780 
KWYYYYQSAN SSSMNGLSSL RKRGRGRPKG RGRGRPRGRP RGSKRINNWD RGPSLIKFKT 

       790        800        810        820        830        840 
GTAILKDYYL KHKFLNEQDL DELVNKSHMG YEQVREWFAE RQRRSELGIE LFEENEEEDE 

       850        860        870 
VIDDQEEDEE ETDDSDTWEP PRHVKRKLSK SDD 

« Hide

Isoform 2 (ZHX1-C8orf76) [UniParc].

See Q96EF9.

References

« Hide 'large scale' references
[1]"Human ZHX1: cloning, chromosomal location, and interaction with transcription factor NF-Y."
Yamada K., Printz R.L., Osawa H., Granner D.K.
Biochem. Biophys. Res. Commun. 261:614-621(1999) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), TISSUE SPECIFICITY, INTERACTION WITH NFYA.
Tissue: Liver.
[2]"Identification and cloning of the human ZHX1 gene."
Mueller R., Ziegler B.L.
Submitted (OCT-1999) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
[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] (ISOFORM 1).
Tissue: Testis.
[4]"Identification of proteins that interact with NF-YA."
Yamada K., Osawa H., Granner D.K.
FEBS Lett. 460:41-45(1999) [PubMed] [Europe PMC] [Abstract]
Cited for: INTERACTION WITH NFYA.
[5]"Functional analysis and the molecular dissection of zinc-fingers and homeoboxes 1 (ZHX1)."
Yamada K., Kawata H., Matsuura K., Shou Z., Hirano S., Mizutani T., Yazawa T., Yoshino M., Sekiguchi T., Kajitani T., Miyamoto K.
Biochem. Biophys. Res. Commun. 297:368-374(2002) [PubMed] [Europe PMC] [Abstract]
Cited for: FUNCTION, HOMODIMERIZATION, SUBCELLULAR LOCATION.
[6]"Analysis of zinc-fingers and homeoboxes (ZHX)-1-interacting proteins: molecular cloning and characterization of a member of the ZHX family, ZHX3."
Yamada K., Kawata H., Shou Z., Hirano S., Mizutani T., Yazawa T., Sekiguchi T., Yoshino M., Kajitani T., Miyamoto K.
Biochem. J. 373:167-178(2003) [PubMed] [Europe PMC] [Abstract]
Cited for: HETERODIMERIZATION WITH ZHX3, INTERACTION WITH ATF7IP.
[7]"Zinc-fingers and homeoboxes (ZHX) 2, a novel member of the ZHX family, functions as a transcriptional repressor."
Kawata H., Yamada K., Shou Z., Mizutani T., Yazawa T., Yoshino M., Sekiguchi T., Kajitani T., Miyamoto K.
Biochem. J. 373:747-757(2003) [PubMed] [Europe PMC] [Abstract]
Cited for: HETERODIMERIZATION WITH ZHX2.
[8]"ZHX proteins regulate podocyte gene expression during the development of nephrotic syndrome."
Liu G., Clement L.C., Kanwar Y.S., Avila-Casado C., Chugh S.S.
J. Biol. Chem. 281:39681-39692(2006) [PubMed] [Europe PMC] [Abstract]
Cited for: SUBCELLULAR LOCATION, TISSUE SPECIFICITY.
Tissue: Kidney.
[9]"A probability-based approach for high-throughput protein phosphorylation analysis and site localization."
Beausoleil S.A., Villen J., Gerber S.A., Rush J., Gygi S.P.
Nat. Biotechnol. 24:1285-1292(2006) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
Tissue: Cervix carcinoma.
[10]"Zinc-fingers and homeoboxes 1 (ZHX1) binds DNA methyltransferase (DNMT) 3B to enhance DNMT3B-mediated transcriptional repression."
Kim S.H., Park J., Choi M.C., Kim H.P., Park J.H., Jung Y., Lee J.H., Oh D.Y., Im S.A., Bang Y.J., Kim T.Y.
Biochem. Biophys. Res. Commun. 355:318-323(2007) [PubMed] [Europe PMC] [Abstract]
Cited for: INTERACTION WITH DNMT3B.
[11]"A quantitative atlas of mitotic phosphorylation."
Dephoure N., Zhou C., Villen J., Beausoleil S.A., Bakalarski C.E., Elledge S.J., Gygi S.P.
Proc. Natl. Acad. Sci. U.S.A. 105:10762-10767(2008) [PubMed] [Europe PMC] [Abstract]
Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-45; SER-47 AND SER-48, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
Tissue: Cervix carcinoma.
[12]"Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach."
Gauci S., Helbig A.O., Slijper M., Krijgsveld J., Heck A.J., Mohammed S.
Anal. Chem. 81:4493-4501(2009) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
[13]"Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions."
Mayya V., Lundgren D.H., Hwang S.-I., Rezaul K., Wu L., Eng J.K., Rodionov V., Han D.K.
Sci. Signal. 2:RA46-RA46(2009) [PubMed] [Europe PMC] [Abstract]
Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-47; SER-48 AND SER-648, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
Tissue: Leukemic T-cell.
[14]"Solution structure of the zinc-finger region of human zinc-fingers and homeoboxes 1 (ZHX1)."
Structural proteomics in Europe (SPINE)
Submitted (APR-2006) to the PDB data bank
Cited for: STRUCTURE BY NMR OF 60-153.
[15]"The solution structure of the third homeobox domain of human zinc fingers and homeoboxes protein."
RIKEN structural genomics initiative (RSGI)
Submitted (FEB-2007) to the PDB data bank
Cited for: STRUCTURE BY NMR OF 565-640.
[16]"The tandem zinc-finger region of human ZHX adopts a novel C2H2 zinc finger structure with a C-terminal extension."
Wienk H., Lammers I., Hotze A., Wu J., Wechselberger R.W., Owens R., Stammers D.K., Stuart D., Kaptein R., Folkers G.E.
Biochemistry 48:4431-4439(2009) [PubMed] [Europe PMC] [Abstract]
Cited for: STRUCTURE BY NMR OF 60-153.
[17]"Novel structural features in two ZHX homeodomains derived from a systematic study of single and multiple domains."
Bird L.E., Ren J., Nettleship J.E., Folkers G.E., Owens R.J., Stammers D.K.
BMC Struct. Biol. 10:13-13(2010) [PubMed] [Europe PMC] [Abstract]
Cited for: X-RAY CRYSTALLOGRAPHY (2.6 ANGSTROMS) OF 655-731.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
AF106862 mRNA. Translation: AAD50624.1.
AF195766 mRNA. Translation: AAF35183.1.
BC040481 mRNA. Translation: AAH40481.1.
PIRJC7079.
RefSeqNP_001017926.1. NM_001017926.2.
NP_009153.3. NM_007222.4.
UniGeneHs.521800.

3D structure databases

PDBe
RCSB PDB
PDBj
EntryMethodResolution (Å)ChainPositionsPDBsum
2ECBNMR-A565-640[»]
2GHFNMR-A60-153[»]
2LY9NMR-A462-532[»]
3NARX-ray2.60A/B655-731[»]
ProteinModelPortalQ9UKY1.
SMRQ9UKY1. Positions 60-153, 296-338, 462-532, 565-637, 662-731, 783-825.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid116406. 55 interactions.
IntActQ9UKY1. 56 interactions.
MINTMINT-1424551.
STRING9606.ENSP00000297857.

PTM databases

PhosphoSiteQ9UKY1.

Polymorphism databases

DMDM44888551.

Proteomic databases

PaxDbQ9UKY1.
PRIDEQ9UKY1.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000297857; ENSP00000297857; ENSG00000165156. [Q9UKY1-1]
ENST00000395571; ENSP00000378938; ENSG00000165156. [Q9UKY1-1]
ENST00000522655; ENSP00000428821; ENSG00000165156. [Q9UKY1-1]
GeneID11244.
KEGGhsa:11244.
UCSCuc003yqe.3. human. [Q9UKY1-1]

Organism-specific databases

CTD11244.
GeneCardsGC08M124260.
HGNCHGNC:12871. ZHX1.
HPAHPA051900.
HPA055357.
MIM604764. gene.
neXtProtNX_Q9UKY1.
PharmGKBPA37460.
GenAtlasSearch...

Phylogenomic databases

eggNOGNOG39488.
HOGENOMHOG000013231.
HOVERGENHBG007920.
InParanoidQ9UKY1.
OMASHMGYEQ.
OrthoDBEOG76HQ0T.
PhylomeDBQ9UKY1.
TreeFamTF333363.

Gene expression databases

ArrayExpressQ9UKY1.
BgeeQ9UKY1.
CleanExHS_ZHX1.
GenevestigatorQ9UKY1.

Family and domain databases

Gene3D1.10.10.60. 5 hits.
InterProIPR001356. Homeobox_dom.
IPR009057. Homeodomain-like.
IPR007087. Znf_C2H2.
IPR015880. Znf_C2H2-like.
[Graphical view]
PfamPF00046. Homeobox. 4 hits.
[Graphical view]
SMARTSM00389. HOX. 5 hits.
SM00355. ZnF_C2H2. 2 hits.
[Graphical view]
SUPFAMSSF46689. SSF46689. 5 hits.
PROSITEPS50071. HOMEOBOX_2. 4 hits.
PS50157. ZINC_FINGER_C2H2_2. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

EvolutionaryTraceQ9UKY1.
GeneWikiZHX1.
GenomeRNAi11244.
NextBio42786.
PROQ9UKY1.
SOURCESearch...

Entry information

Entry nameZHX1_HUMAN
AccessionPrimary (citable) accession number: Q9UKY1
Secondary accession number(s): Q8IWD8
Entry history
Integrated into UniProtKB/Swiss-Prot: March 1, 2004
Last sequence update: May 1, 2000
Last modified: April 16, 2014
This is version 122 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

PDB cross-references

Index of Protein Data Bank (PDB) cross-references

MIM cross-references

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

Human chromosome 8

Human chromosome 8: entries, gene names and cross-references to MIM