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Protein

Zinc fingers and homeoboxes protein 1

Gene

ZHX1

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

Functioni

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.1 Publication

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri70 – 93C2H2-type 1PROSITE-ProRule annotationCuratedAdd BLAST24
Zinc fingeri102 – 125C2H2-type 2PROSITE-ProRule annotationCuratedAdd BLAST24
DNA bindingi284 – 346Homeobox 1PROSITE-ProRule annotationCuratedAdd BLAST63
DNA bindingi464 – 526Homeobox 2PROSITE-ProRule annotationCuratedAdd BLAST63
DNA bindingi569 – 630Homeobox 3PROSITE-ProRule annotationCuratedAdd BLAST62
DNA bindingi660 – 722Homeobox 4PROSITE-ProRule annotationCuratedAdd BLAST63
DNA bindingi777 – 832Homeobox 5PROSITE-ProRule annotationCuratedAdd BLAST56

GO - Molecular functioni

  • DNA binding Source: UniProtKB-KW
  • metal ion binding Source: UniProtKB-KW
  • protein heterodimerization activity Source: UniProtKB
  • transcription corepressor activity Source: UniProtKB
  • transcription factor activity, sequence-specific DNA binding Source: UniProtKB

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Repressor

Keywords - Biological processi

Differentiation, Transcription, Transcription regulation

Keywords - Ligandi

DNA-binding, Metal-binding, Zinc

Names & Taxonomyi

Protein namesi
Recommended name:
Zinc fingers and homeoboxes protein 1
Gene namesi
Name:ZHX1
OrganismiHomo sapiens (Human)Imported
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 8

Organism-specific databases

HGNCiHGNC:12871. ZHX1.

Subcellular locationi

GO - Cellular componenti

  • nucleoplasm Source: HPA
  • nucleus Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Nucleus

Pathology & Biotechi

Organism-specific databases

DisGeNETi11244.
OpenTargetsiENSG00000165156.
PharmGKBiPA37460.

Polymorphism and mutation databases

BioMutaiZHX1.
DMDMi44888551.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000493881 – 873Zinc fingers and homeoboxes protein 1Add BLAST873

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei36PhosphothreonineCombined sources1
Modified residuei45PhosphoserineCombined sources1
Modified residuei47PhosphoserineCombined sources1
Modified residuei48PhosphoserineCombined sources1
Modified residuei202PhosphoserineCombined sources1
Cross-linki454Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Modified residuei648PhosphoserineCombined sources1
Modified residuei774PhosphoserineCombined sources1

Keywords - PTMi

Isopeptide bond, Phosphoprotein, Ubl conjugation

Proteomic databases

EPDiQ9UKY1.
MaxQBiQ9UKY1.
PaxDbiQ9UKY1.
PeptideAtlasiQ9UKY1.
PRIDEiQ9UKY1.

PTM databases

iPTMnetiQ9UKY1.
PhosphoSitePlusiQ9UKY1.

Expressioni

Tissue specificityi

Ubiquitously expressed. Expressed in podocytes.2 Publications

Gene expression databases

BgeeiENSG00000165156.
CleanExiHS_ZHX1.
ExpressionAtlasiQ9UKY1. baseline and differential.
GenevisibleiQ9UKY1. HS.

Organism-specific databases

HPAiHPA051900.
HPA055357.

Interactioni

Subunit structurei

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).4 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
ATXN1P542535EBI-347767,EBI-930964
DNMT3BQ9UBC3-16EBI-347767,EBI-6083193
ZHX2Q9Y6X82EBI-347767,EBI-948566

GO - Molecular functioni

  • protein heterodimerization activity Source: UniProtKB

Protein-protein interaction databases

BioGridi116406. 58 interactors.
IntActiQ9UKY1. 58 interactors.
MINTiMINT-1424551.
STRINGi9606.ENSP00000297857.

Structurei

Secondary structure

1873
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Turni60 – 62Combined sources3
Beta strandi68 – 71Combined sources4
Beta strandi78 – 80Combined sources3
Helixi82 – 92Combined sources11
Beta strandi102 – 104Combined sources3
Turni105 – 108Combined sources4
Beta strandi109 – 113Combined sources5
Helixi115 – 118Combined sources4
Helixi120 – 123Combined sources4
Beta strandi131 – 135Combined sources5
Beta strandi144 – 150Combined sources7
Helixi473 – 485Combined sources13
Helixi491 – 501Combined sources11
Helixi505 – 518Combined sources14
Turni519 – 523Combined sources5
Helixi577 – 589Combined sources13
Helixi595 – 604Combined sources10
Helixi609 – 624Combined sources16
Beta strandi664 – 667Combined sources4
Helixi669 – 681Combined sources13
Helixi687 – 697Combined sources11
Helixi701 – 715Combined sources15
Turni716 – 718Combined sources3
Helixi721 – 730Combined sources10

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2ECBNMR-A565-640[»]
2GHFNMR-A60-153[»]
2LY9NMR-A462-532[»]
3NARX-ray2.60A/B655-731[»]
ProteinModelPortaliQ9UKY1.
SMRiQ9UKY1.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ9UKY1.

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni272 – 564Required for interaction with NFYAAdd BLAST293
Regioni272 – 432Required for dimerizationAdd BLAST161
Regioni734 – 768Required for nuclear localizationAdd BLAST35
Regioni831 – 873Required for repressor activityAdd BLAST43

Sequence similaritiesi

Belongs to the ZHX family.Curated
Contains 2 C2H2-type zinc fingers.PROSITE-ProRule annotationCurated
Contains 5 homeobox DNA-binding domains.PROSITE-ProRule annotation

Zinc finger

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri70 – 93C2H2-type 1PROSITE-ProRule annotationCuratedAdd BLAST24
Zinc fingeri102 – 125C2H2-type 2PROSITE-ProRule annotationCuratedAdd BLAST24

Keywords - Domaini

Homeobox, Repeat, Zinc-finger

Phylogenomic databases

eggNOGiENOG410IHET. Eukaryota.
ENOG410Z8A2. LUCA.
GeneTreeiENSGT00530000063149.
HOGENOMiHOG000013231.
HOVERGENiHBG007920.
InParanoidiQ9UKY1.
OMAiSHMGYEQ.
OrthoDBiEOG091G02WT.
PhylomeDBiQ9UKY1.
TreeFamiTF333363.

Family and domain databases

Gene3Di1.10.10.60. 5 hits.
InterProiIPR001356. Homeobox_dom.
IPR009057. Homeodomain-like.
IPR024578. Homez_dom.
IPR007087. Znf_C2H2.
IPR015880. Znf_C2H2-like.
[Graphical view]
PfamiPF00046. Homeobox. 4 hits.
PF11569. Homez. 1 hit.
[Graphical view]
SMARTiSM00389. HOX. 5 hits.
SM00355. ZnF_C2H2. 2 hits.
[Graphical view]
SUPFAMiSSF46689. SSF46689. 5 hits.
PROSITEiPS50071. HOMEOBOX_2. 4 hits.
PS50157. ZINC_FINGER_C2H2_2. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q9UKY1-1) [UniParc]FASTAAdd to basket

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.

« Hide

        10         20         30         40         50
MASRRKSTTP CMVLASEQDP DLELISDLDE GPPVLTPVEN TRAESISSDE
60 70 80 90 100
EVHESVDSDN QQNKKVEGGY ECKYCTFQTP DLNMFTFHVD SEHPNVVLNS
110 120 130 140 150
SYVCVECNFL TKRYDALSEH NLKYHPGEEN FKLTMVKRNN QTIFEQTIND
160 170 180 190 200
LTFDGSFVKE ENAEQAESTE VSSSGISISK TPIMKMMKNK VENKRIAVHH
210 220 230 240 250
NSVEDVPEEK ENEIKPDREE IVENPSSSAS ESNTSTSIVN RIHPSTASTV
260 270 280 290 300
VTPAAVLPGL AQVITAVSAQ QNSNLIPKVL IPVNSIPTYN AALDNNPLLL
310 320 330 340 350
NTYNKFPYPT MSEITVLSAQ AKYTEEQIKI WFSAQRLKHG VSWTPEEVEE
360 370 380 390 400
ARRKQFNGTV HTVPQTITVI PTHISTGSNG LPSILQTCQI VGQPGLVLTQ
410 420 430 440 450
VAGTNTLPVT APIALTVAGV PSQNNIQKSQ VPAAQPTAET KPATAAVPTS
460 470 480 490 500
QSVKHETALV NPDSFGIRAK KTKEQLAELK VSYLKNQFPH DSEIIRLMKI
510 520 530 540 550
TGLTKGEIKK WFSDTRYNQR NSKSNQCLHL NNDSSTTIII DSSDETTESP
560 570 580 590 600
TVGTAQPKQS WNPFPDFTPQ KFKEKTAEQL RVLQASFLNS SVLTDEELNR
610 620 630 640 650
LRAQTKLTRR EIDAWFTEKK KSKALKEEKM EIDESNAGSS KEEAGETSPA
660 670 680 690 700
DESGAPKSGS TGKICKKTPE QLHMLKSAFV RTQWPSPEEY DKLAKESGLA
710 720 730 740 750
RTDIVSWFGD TRYAWKNGNL KWYYYYQSAN SSSMNGLSSL RKRGRGRPKG
760 770 780 790 800
RGRGRPRGRP RGSKRINNWD RGPSLIKFKT GTAILKDYYL KHKFLNEQDL
810 820 830 840 850
DELVNKSHMG YEQVREWFAE RQRRSELGIE LFEENEEEDE VIDDQEEDEE
860 870
ETDDSDTWEP PRHVKRKLSK SDD
Length:873
Mass (Da):98,098
Last modified:May 1, 2000 - v1
Checksum:i66CF1CEC5EF824E5
GO
Isoform 2 (identifier: Q96EF9-1) [UniParc]FASTAAdd to basket
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.
Length:292
Mass (Da):33,285
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti263V → M in AAH40481 (PubMed:15489334).Curated1
Sequence conflicti792H → R in AAH40481 (PubMed:15489334).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF106862 mRNA. Translation: AAD50624.1.
AF195766 mRNA. Translation: AAF35183.1.
BC040481 mRNA. Translation: AAH40481.1.
CCDSiCCDS6342.1. [Q9UKY1-1]
PIRiJC7079.
RefSeqiNP_001017926.1. NM_001017926.2. [Q9UKY1-1]
NP_009153.3. NM_007222.4. [Q9UKY1-1]
UniGeneiHs.521800.

Genome annotation databases

EnsembliENST00000297857; ENSP00000297857; ENSG00000165156. [Q9UKY1-1]
ENST00000395571; ENSP00000378938; ENSG00000165156. [Q9UKY1-1]
ENST00000522655; ENSP00000428821; ENSG00000165156. [Q9UKY1-1]
GeneIDi11244.
KEGGihsa:11244.
UCSCiuc003yqe.4. human. [Q9UKY1-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF106862 mRNA. Translation: AAD50624.1.
AF195766 mRNA. Translation: AAF35183.1.
BC040481 mRNA. Translation: AAH40481.1.
CCDSiCCDS6342.1. [Q9UKY1-1]
PIRiJC7079.
RefSeqiNP_001017926.1. NM_001017926.2. [Q9UKY1-1]
NP_009153.3. NM_007222.4. [Q9UKY1-1]
UniGeneiHs.521800.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2ECBNMR-A565-640[»]
2GHFNMR-A60-153[»]
2LY9NMR-A462-532[»]
3NARX-ray2.60A/B655-731[»]
ProteinModelPortaliQ9UKY1.
SMRiQ9UKY1.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi116406. 58 interactors.
IntActiQ9UKY1. 58 interactors.
MINTiMINT-1424551.
STRINGi9606.ENSP00000297857.

PTM databases

iPTMnetiQ9UKY1.
PhosphoSitePlusiQ9UKY1.

Polymorphism and mutation databases

BioMutaiZHX1.
DMDMi44888551.

Proteomic databases

EPDiQ9UKY1.
MaxQBiQ9UKY1.
PaxDbiQ9UKY1.
PeptideAtlasiQ9UKY1.
PRIDEiQ9UKY1.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000297857; ENSP00000297857; ENSG00000165156. [Q9UKY1-1]
ENST00000395571; ENSP00000378938; ENSG00000165156. [Q9UKY1-1]
ENST00000522655; ENSP00000428821; ENSG00000165156. [Q9UKY1-1]
GeneIDi11244.
KEGGihsa:11244.
UCSCiuc003yqe.4. human. [Q9UKY1-1]

Organism-specific databases

CTDi11244.
DisGeNETi11244.
GeneCardsiZHX1.
HGNCiHGNC:12871. ZHX1.
HPAiHPA051900.
HPA055357.
MIMi604764. gene.
neXtProtiNX_Q9UKY1.
OpenTargetsiENSG00000165156.
PharmGKBiPA37460.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IHET. Eukaryota.
ENOG410Z8A2. LUCA.
GeneTreeiENSGT00530000063149.
HOGENOMiHOG000013231.
HOVERGENiHBG007920.
InParanoidiQ9UKY1.
OMAiSHMGYEQ.
OrthoDBiEOG091G02WT.
PhylomeDBiQ9UKY1.
TreeFamiTF333363.

Miscellaneous databases

EvolutionaryTraceiQ9UKY1.
GeneWikiiZHX1.
GenomeRNAii11244.
PROiQ9UKY1.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000165156.
CleanExiHS_ZHX1.
ExpressionAtlasiQ9UKY1. baseline and differential.
GenevisibleiQ9UKY1. HS.

Family and domain databases

Gene3Di1.10.10.60. 5 hits.
InterProiIPR001356. Homeobox_dom.
IPR009057. Homeodomain-like.
IPR024578. Homez_dom.
IPR007087. Znf_C2H2.
IPR015880. Znf_C2H2-like.
[Graphical view]
PfamiPF00046. Homeobox. 4 hits.
PF11569. Homez. 1 hit.
[Graphical view]
SMARTiSM00389. HOX. 5 hits.
SM00355. ZnF_C2H2. 2 hits.
[Graphical view]
SUPFAMiSSF46689. SSF46689. 5 hits.
PROSITEiPS50071. HOMEOBOX_2. 4 hits.
PS50157. ZINC_FINGER_C2H2_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiZHX1_HUMAN
AccessioniPrimary (citable) accession number: Q9UKY1
Secondary accession number(s): Q8IWD8
Entry historyi
Integrated into UniProtKB/Swiss-Prot: March 1, 2004
Last sequence update: May 1, 2000
Last modified: November 2, 2016
This is version 147 of the entry and version 1 of the sequence. [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

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 8
    Human chromosome 8: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  4. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.