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Protein

Z-DNA-binding protein 1

Gene

ZBP1

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

Functioni

Participates in the detection by the host's innate immune system of DNA from viral, bacterial or even host origin. Plays a role in host defense against tumors and pathogens. Acts as a cytoplasmic DNA sensor which, when activated, induces the recruitment of TBK1 and IRF3 to its C-terminal region and activates the downstream interferon regulatory factor (IRF) and NF-kappa B transcription factors, leading to type-I interferon production. ZBP1-induced NF-kappaB activation probably involves the recruitment of the RHIM containing kinases RIPK1 and RIPK3 (By similarity).By similarity

GO - Molecular functioni

  • DNA binding Source: MGI
  • double-stranded RNA adenosine deaminase activity Source: InterPro
  • identical protein binding Source: IntAct
  • left-handed Z-DNA binding Source: UniProtKB
  • RNA binding Source: InterPro

GO - Biological processi

Keywordsi

Molecular functionDNA-binding
Biological processImmunity, Innate immunity

Enzyme and pathway databases

ReactomeiR-HSA-1606322 ZBP1(DAI) mediated induction of type I IFNs
R-HSA-1606341 IRF3 mediated activation of type 1 IFN
R-HSA-1810476 RIP-mediated NFkB activation via ZBP1
R-HSA-3134975 Regulation of innate immune responses to cytosolic DNA

Names & Taxonomyi

Protein namesi
Recommended name:
Z-DNA-binding protein 1
Alternative name(s):
Tumor stroma and activated macrophage protein DLM-1
Gene namesi
Name:ZBP1
Synonyms:C20orf183, DLM1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 20

Organism-specific databases

EuPathDBiHostDB:ENSG00000124256.14
HGNCiHGNC:16176 ZBP1
MIMi606750 gene
neXtProtiNX_Q9H171

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

Pathology & Biotechi

Organism-specific databases

DisGeNETi81030
OpenTargetsiENSG00000124256
PharmGKBiPA38094

Polymorphism and mutation databases

BioMutaiZBP1
DMDMi71153189

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000665641 – 429Z-DNA-binding protein 1Add BLAST429

Proteomic databases

PaxDbiQ9H171
PeptideAtlasiQ9H171
PRIDEiQ9H171
ProteomicsDBi80370
80371 [Q9H171-2]
80372 [Q9H171-3]
80373 [Q9H171-4]
80374 [Q9H171-5]

PTM databases

iPTMnetiQ9H171
PhosphoSitePlusiQ9H171

Expressioni

Tissue specificityi

Highly expressed in lymphatic tissues including lymph node, leukocytes, tonsil, bone marrow and spleen. Expressed to a lesser extent in thymus, lung and liver.

Gene expression databases

BgeeiENSG00000124256 Expressed in 145 organ(s), highest expression level in blood
CleanExiHS_ZBP1
ExpressionAtlasiQ9H171 baseline and differential
GenevisibleiQ9H171 HS

Organism-specific databases

HPAiHPA041256

Interactioni

Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

BioGridi123349, 13 interactors
DIPiDIP-44121N
IntActiQ9H171, 6 interactors
MINTiQ9H171
STRINGi9606.ENSP00000360215

Structurei

Secondary structure

1429
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliQ9H171
SMRiQ9H171
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ9H171

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Repeati6 – 72DRADA 1Add BLAST67
Repeati103 – 168DRADA 2Add BLAST66

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi195 – 219RIP homotypic interaction motif (RHIM) 1Add BLAST25
Motifi253 – 277RIP homotypic interaction motif (RHIM) 2Add BLAST25

Keywords - Domaini

Repeat

Phylogenomic databases

eggNOGiENOG410J2S4 Eukaryota
ENOG411196S LUCA
GeneTreeiENSGT00390000002234
HOGENOMiHOG000230878
HOVERGENiHBG036279
InParanoidiQ9H171
KOiK12965
OMAiKECQVPK
OrthoDBiEOG091G0L53
PhylomeDBiQ9H171
TreeFamiTF337658

Family and domain databases

Gene3Di1.10.10.10, 2 hits
InterProiView protein in InterPro
IPR000607 dsRNA_A_deaminase
IPR036388 WH-like_DNA-bd_sf
IPR036390 WH_DNA-bd_sf
PfamiView protein in Pfam
PF02295 z-alpha, 1 hit
SMARTiView protein in SMART
SM00550 Zalpha, 2 hits
SUPFAMiSSF46785 SSF46785, 2 hits

Sequences (7+)i

Sequence statusi: Complete.

This entry describes 7 isoformsi produced by alternative splicing. AlignAdd to basket
Note: Additional isoforms seem to exist.

This entry has 7 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All

Isoform 1 (identifier: Q9H171-1) [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
MAQAPADPGR EGHLEQRILQ VLTEAGSPVK LAQLVKECQA PKRELNQVLY
60 70 80 90 100
RMKKELKVSL TSPATWCLGG TDPEGEGPAE LALSSPAERP QQHAATIPET
110 120 130 140 150
PGPQFSQQRE EDIYRFLKDN GPQRALVIAQ ALGMRTAKDV NRDLYRMKSR
160 170 180 190 200
HLLDMDEQSK AWTIYRPEDS GRRAKSASII YQHNPINMIC QNGPNSWISI
210 220 230 240 250
ANSEAIQIGH GNIITRQTVS REDGSAGPRH LPSMAPGDSS TWGTLVDPWG
260 270 280 290 300
PQDIHMEQSI LRRVQLGHSN EMRLHGVPSE GPAHIPPGSP PVSATAAGPE
310 320 330 340 350
ASFEARIPSP GTHPEGEAAQ RIHMKSCFLE DATIGNSNKM SISPGVAGPG
360 370 380 390 400
GVAGSGEGEP GEDAGRRPAD TQSRSHFPRD IGQPITPSHS KLTPKLETMT
410 420
LGNRSHKAAE GSHYVDEASH EGSWWGGGI
Length:429
Mass (Da):46,343
Last modified:July 19, 2005 - v2
Checksum:i2C9B195112822991
GO
Isoform 2 (identifier: Q9H171-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     365-429: GRRPADTQSR...HEGSWWGGGI → VAAFTEVLNP...HKIYLLKNKK

Show »
Length:446
Mass (Da):48,754
Checksum:iAB6807A7F1397076
GO
Isoform 3 (identifier: Q9H171-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     365-429: GRRPADTQSR...HEGSWWGGGI → GRRPADTQSRSFDGQE

Show »
Length:380
Mass (Da):41,028
Checksum:i247472C6F85CEB36
GO
Isoform 4 (identifier: Q9H171-4) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     87-109: Missing.

Show »
Length:406
Mass (Da):43,786
Checksum:i709506D06777E8F2
GO
Isoform 5 (identifier: Q9H171-5) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     87-149: AERPQQHAAT...VNRDLYRMKS → GNCHPGEAGL...LTAGLLGRGC
     150-429: Missing.

Show »
Length:149
Mass (Da):15,985
Checksum:i1D948BB00599314B
GO
Isoform 6 (identifier: Q9H171-6) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     225-248: SAGPRHLPSMAPGDSSTWGTLVDP → KSPKRAQGGDLGGEPPDPLGGGKG
     249-429: Missing.

Note: No experimental confirmation available.
Show »
Length:248
Mass (Da):27,218
Checksum:iE79D75F5D4FE665C
GO
Isoform 7 (identifier: Q9H171-7) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     12-86: Missing.

Note: No experimental confirmation available.
Show »
Length:354
Mass (Da):38,220
Checksum:i162A79FBD15B1B0F
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H0Y6D1H0Y6D1_HUMAN
Z-DNA-binding protein 1
ZBP1
119Annotation score:

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05703017R → I. Corresponds to variant dbSNP:rs35813125Ensembl.1
Natural variantiVAR_06172853K → R. Corresponds to variant dbSNP:rs35895307Ensembl.1
Natural variantiVAR_05703170G → R. Corresponds to variant dbSNP:rs34964609Ensembl.1
Natural variantiVAR_01431688E → K2 PublicationsCorresponds to variant dbSNP:rs2073145Ensembl.1
Natural variantiVAR_057032154D → H. Corresponds to variant dbSNP:rs16981187Ensembl.1
Natural variantiVAR_057033166R → H. Corresponds to variant dbSNP:rs34478944Ensembl.1
Natural variantiVAR_069138258Q → R3 PublicationsCorresponds to variant dbSNP:rs2865394Ensembl.1
Natural variantiVAR_061729332A → V. Corresponds to variant dbSNP:rs41275648Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_04608112 – 86Missing in isoform 7. 1 PublicationAdd BLAST75
Alternative sequenceiVSP_00407987 – 149AERPQ…YRMKS → GNCHPGEAGLTLQGASWQWT STDLSLGSNLNSATWELTGF LSLCLGFFFWLMELTAGLLG RGC in isoform 5. 1 PublicationAdd BLAST63
Alternative sequenceiVSP_00407887 – 109Missing in isoform 4. CuratedAdd BLAST23
Alternative sequenceiVSP_004080150 – 429Missing in isoform 5. 1 PublicationAdd BLAST280
Alternative sequenceiVSP_045405225 – 248SAGPR…TLVDP → KSPKRAQGGDLGGEPPDPLG GGKG in isoform 6. 1 PublicationAdd BLAST24
Alternative sequenceiVSP_045406249 – 429Missing in isoform 6. 1 PublicationAdd BLAST181
Alternative sequenceiVSP_004081365 – 429GRRPA…WGGGI → VAAFTEVLNPSKSFMKFGIN FFQTPVNVDSLTSSHESQMF LTACRLVIFSRRFSTYFAQI HLRNHSVLHLCPHKIYLLKN KK in isoform 2. CuratedAdd BLAST65
Alternative sequenceiVSP_004082365 – 429GRRPA…WGGGI → GRRPADTQSRSFDGQE in isoform 3. CuratedAdd BLAST65

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ300575 mRNA Translation: CAC18810.1
CT002036 mRNA No translation available.
AK122761 mRNA Translation: BAG53713.1
AL035541 Genomic DNA No translation available.
CH471077 Genomic DNA Translation: EAW75511.1
BC028218 mRNA Translation: AAH28218.1
CCDSiCCDS13461.1 [Q9H171-1]
CCDS54477.1 [Q9H171-7]
CCDS54478.1 [Q9H171-6]
RefSeqiNP_001153889.1, NM_001160417.1
NP_001153890.1, NM_001160418.1 [Q9H171-7]
NP_001153891.1, NM_001160419.2 [Q9H171-6]
NP_001310895.1, NM_001323966.1
NP_110403.2, NM_030776.2 [Q9H171-1]
UniGeneiHs.302123

Genome annotation databases

EnsembliENST00000371173; ENSP00000360215; ENSG00000124256 [Q9H171-1]
ENST00000395822; ENSP00000379167; ENSG00000124256 [Q9H171-7]
ENST00000541799; ENSP00000440552; ENSG00000124256 [Q9H171-6]
GeneIDi81030
KEGGihsa:81030
UCSCiuc002xyo.4 human [Q9H171-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ300575 mRNA Translation: CAC18810.1
CT002036 mRNA No translation available.
AK122761 mRNA Translation: BAG53713.1
AL035541 Genomic DNA No translation available.
CH471077 Genomic DNA Translation: EAW75511.1
BC028218 mRNA Translation: AAH28218.1
CCDSiCCDS13461.1 [Q9H171-1]
CCDS54477.1 [Q9H171-7]
CCDS54478.1 [Q9H171-6]
RefSeqiNP_001153889.1, NM_001160417.1
NP_001153890.1, NM_001160418.1 [Q9H171-7]
NP_001153891.1, NM_001160419.2 [Q9H171-6]
NP_001310895.1, NM_001323966.1
NP_110403.2, NM_030776.2 [Q9H171-1]
UniGeneiHs.302123

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2L4MNMR-A103-166[»]
2LNBNMR-A6-74[»]
3EYIX-ray1.45A/B103-166[»]
4KA4X-ray2.60A/B/D/E96-165[»]
ProteinModelPortaliQ9H171
SMRiQ9H171
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi123349, 13 interactors
DIPiDIP-44121N
IntActiQ9H171, 6 interactors
MINTiQ9H171
STRINGi9606.ENSP00000360215

PTM databases

iPTMnetiQ9H171
PhosphoSitePlusiQ9H171

Polymorphism and mutation databases

BioMutaiZBP1
DMDMi71153189

Proteomic databases

PaxDbiQ9H171
PeptideAtlasiQ9H171
PRIDEiQ9H171
ProteomicsDBi80370
80371 [Q9H171-2]
80372 [Q9H171-3]
80373 [Q9H171-4]
80374 [Q9H171-5]

Protocols and materials databases

DNASUi81030
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000371173; ENSP00000360215; ENSG00000124256 [Q9H171-1]
ENST00000395822; ENSP00000379167; ENSG00000124256 [Q9H171-7]
ENST00000541799; ENSP00000440552; ENSG00000124256 [Q9H171-6]
GeneIDi81030
KEGGihsa:81030
UCSCiuc002xyo.4 human [Q9H171-1]

Organism-specific databases

CTDi81030
DisGeNETi81030
EuPathDBiHostDB:ENSG00000124256.14
GeneCardsiZBP1
H-InvDBiHIX0203057
HGNCiHGNC:16176 ZBP1
HPAiHPA041256
MIMi606750 gene
neXtProtiNX_Q9H171
OpenTargetsiENSG00000124256
PharmGKBiPA38094
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410J2S4 Eukaryota
ENOG411196S LUCA
GeneTreeiENSGT00390000002234
HOGENOMiHOG000230878
HOVERGENiHBG036279
InParanoidiQ9H171
KOiK12965
OMAiKECQVPK
OrthoDBiEOG091G0L53
PhylomeDBiQ9H171
TreeFamiTF337658

Enzyme and pathway databases

ReactomeiR-HSA-1606322 ZBP1(DAI) mediated induction of type I IFNs
R-HSA-1606341 IRF3 mediated activation of type 1 IFN
R-HSA-1810476 RIP-mediated NFkB activation via ZBP1
R-HSA-3134975 Regulation of innate immune responses to cytosolic DNA

Miscellaneous databases

EvolutionaryTraceiQ9H171
GeneWikiiZBP1
GenomeRNAii81030
PROiPR:Q9H171
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000124256 Expressed in 145 organ(s), highest expression level in blood
CleanExiHS_ZBP1
ExpressionAtlasiQ9H171 baseline and differential
GenevisibleiQ9H171 HS

Family and domain databases

Gene3Di1.10.10.10, 2 hits
InterProiView protein in InterPro
IPR000607 dsRNA_A_deaminase
IPR036388 WH-like_DNA-bd_sf
IPR036390 WH_DNA-bd_sf
PfamiView protein in Pfam
PF02295 z-alpha, 1 hit
SMARTiView protein in SMART
SM00550 Zalpha, 2 hits
SUPFAMiSSF46785 SSF46785, 2 hits
ProtoNetiSearch...

Entry informationi

Entry nameiZBP1_HUMAN
AccessioniPrimary (citable) accession number: Q9H171
Secondary accession number(s): A2A2F7
, B3KVA1, F5GYT1, Q5JY39, Q9BYW4
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 25, 2002
Last sequence update: July 19, 2005
Last modified: November 7, 2018
This is version 152 of the entry and version 2 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

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  2. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  3. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  4. Human chromosome 20
    Human chromosome 20: entries, gene names and cross-references to MIM
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
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Main funding by: National Institutes of Health

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