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Q9H171 (ZBP1_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 120. 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·Interactions·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Z-DNA-binding protein 1
Alternative name(s):
Tumor stroma and activated macrophage protein DLM-1
Gene names
Name:ZBP1
Synonyms:C20orf183, DLM1
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length429 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

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.

Tissue specificity

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

Sequence similarities

Contains 2 DRADA repeats.

Binary interactions

Alternative products

This entry describes 7 isoforms produced by alternative splicing. [Align] [Select]

Note: Additional isoforms seem to exist.
Isoform 1 (identifier: Q9H171-1)

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: Q9H171-2)

The sequence of this isoform differs from the canonical sequence as follows:
     365-429: GRRPADTQSR...HEGSWWGGGI → VAAFTEVLNP...HKIYLLKNKK
Isoform 3 (identifier: Q9H171-3)

The sequence of this isoform differs from the canonical sequence as follows:
     365-429: GRRPADTQSR...HEGSWWGGGI → GRRPADTQSRSFDGQE
Isoform 4 (identifier: Q9H171-4)

The sequence of this isoform differs from the canonical sequence as follows:
     87-109: Missing.
Isoform 5 (identifier: Q9H171-5)

The sequence of this isoform differs from the canonical sequence as follows:
     87-149: AERPQQHAAT...VNRDLYRMKS → GNCHPGEAGL...LTAGLLGRGC
     150-429: Missing.
Isoform 6 (identifier: Q9H171-6)

The sequence of this isoform differs from the canonical sequence as follows:
     225-248: SAGPRHLPSMAPGDSSTWGTLVDP → KSPKRAQGGDLGGEPPDPLGGGKG
     249-429: Missing.
Note: No experimental confirmation available.
Isoform 7 (identifier: Q9H171-7)

The sequence of this isoform differs from the canonical sequence as follows:
     12-86: Missing.
Note: No experimental confirmation available.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 429429Z-DNA-binding protein 1
PRO_0000066564

Regions

Repeat6 – 7267DRADA 1
Repeat103 – 16866DRADA 2
Motif195 – 21925RIP homotypic interaction motif (RHIM) 1
Motif253 – 27725RIP homotypic interaction motif (RHIM) 2

Natural variations

Alternative sequence12 – 8675Missing in isoform 7.
VSP_046081
Alternative sequence87 – 14963AERPQ…YRMKS → GNCHPGEAGLTLQGASWQWT STDLSLGSNLNSATWELTGF LSLCLGFFFWLMELTAGLLG RGC in isoform 5.
VSP_004079
Alternative sequence87 – 10923Missing in isoform 4.
VSP_004078
Alternative sequence150 – 429280Missing in isoform 5.
VSP_004080
Alternative sequence225 – 24824SAGPR…TLVDP → KSPKRAQGGDLGGEPPDPLG GGKG in isoform 6.
VSP_045405
Alternative sequence249 – 429181Missing in isoform 6.
VSP_045406
Alternative sequence365 – 42965GRRPA…WGGGI → VAAFTEVLNPSKSFMKFGIN FFQTPVNVDSLTSSHESQMF LTACRLVIFSRRFSTYFAQI HLRNHSVLHLCPHKIYLLKN KK in isoform 2.
VSP_004081
Alternative sequence365 – 42965GRRPA…WGGGI → GRRPADTQSRSFDGQE in isoform 3.
VSP_004082
Natural variant171R → I.
Corresponds to variant rs35813125 [ dbSNP | Ensembl ].
VAR_057030
Natural variant531K → R.
Corresponds to variant rs35895307 [ dbSNP | Ensembl ].
VAR_061728
Natural variant701G → R.
Corresponds to variant rs34964609 [ dbSNP | Ensembl ].
VAR_057031
Natural variant881E → K. Ref.1 Ref.2
Corresponds to variant rs2073145 [ dbSNP | Ensembl ].
VAR_014316
Natural variant1541D → H.
Corresponds to variant rs16981187 [ dbSNP | Ensembl ].
VAR_057032
Natural variant1661R → H.
Corresponds to variant rs34478944 [ dbSNP | Ensembl ].
VAR_057033
Natural variant2581Q → R. Ref.1 Ref.3 Ref.5
Corresponds to variant rs2865394 [ dbSNP | Ensembl ].
VAR_069138
Natural variant3321A → V.
Corresponds to variant rs41275648 [ dbSNP | Ensembl ].
VAR_061729

Secondary structure

.......................... 429
Helix Strand Turn

Details...

Sequences

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

Last modified July 19, 2005. Version 2.
Checksum: 2C9B195112822991

FASTA42946,343
        10         20         30         40         50         60 
MAQAPADPGR EGHLEQRILQ VLTEAGSPVK LAQLVKECQA PKRELNQVLY RMKKELKVSL 

        70         80         90        100        110        120 
TSPATWCLGG TDPEGEGPAE LALSSPAERP QQHAATIPET PGPQFSQQRE EDIYRFLKDN 

       130        140        150        160        170        180 
GPQRALVIAQ ALGMRTAKDV NRDLYRMKSR HLLDMDEQSK AWTIYRPEDS GRRAKSASII 

       190        200        210        220        230        240 
YQHNPINMIC QNGPNSWISI ANSEAIQIGH GNIITRQTVS REDGSAGPRH LPSMAPGDSS 

       250        260        270        280        290        300 
TWGTLVDPWG PQDIHMEQSI LRRVQLGHSN EMRLHGVPSE GPAHIPPGSP PVSATAAGPE 

       310        320        330        340        350        360 
ASFEARIPSP GTHPEGEAAQ RIHMKSCFLE DATIGNSNKM SISPGVAGPG GVAGSGEGEP 

       370        380        390        400        410        420 
GEDAGRRPAD TQSRSHFPRD IGQPITPSHS KLTPKLETMT LGNRSHKAAE GSHYVDEASH 


EGSWWGGGI 

« Hide

Isoform 2 [UniParc].

Checksum: AB6807A7F1397076
Show »

FASTA44648,754
Isoform 3 [UniParc].

Checksum: 247472C6F85CEB36
Show »

FASTA38041,028
Isoform 4 [UniParc].

Checksum: 709506D06777E8F2
Show »

FASTA40643,786
Isoform 5 [UniParc].

Checksum: 1D948BB00599314B
Show »

FASTA14915,985
Isoform 6 [UniParc].

Checksum: E79D75F5D4FE665C
Show »

FASTA24827,218
Isoform 7 [UniParc].

Checksum: 162A79FBD15B1B0F
Show »

FASTA35438,220

References

« Hide 'large scale' references
[1]"Complex regulation of the human gene for the Z-DNA binding protein DLM-1."
Rothenburg S., Schwartz T., Koch-Nolte F., Haag F.
Nucleic Acids Res. 30:993-1000(2002) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA], ALTERNATIVE SPLICING, VARIANTS LYS-88 AND ARG-258.
Tissue: Spleen.
[2]Heil O., Ebert L., Hennig S., Henze S., Radelof U., Schneider D., Korn B.
Submitted (JUN-2005) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 6), VARIANT LYS-88.
Tissue: T-cell.
[3]"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S. expand/collapse author list , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 7), VARIANT ARG-258.
[4]"The DNA sequence and comparative analysis of human chromosome 20."
Deloukas P., Matthews L.H., Ashurst J.L., Burton J., Gilbert J.G.R., Jones M., Stavrides G., Almeida J.P., Babbage A.K., Bagguley C.L., Bailey J., Barlow K.F., Bates K.N., Beard L.M., Beare D.M., Beasley O.P., Bird C.P., Blakey S.E. expand/collapse author list , Bridgeman A.M., Brown A.J., Buck D., Burrill W.D., Butler A.P., Carder C., Carter N.P., Chapman J.C., Clamp M., Clark G., Clark L.N., Clark S.Y., Clee C.M., Clegg S., Cobley V.E., Collier R.E., Connor R.E., Corby N.R., Coulson A., Coville G.J., Deadman R., Dhami P.D., Dunn M., Ellington A.G., Frankland J.A., Fraser A., French L., Garner P., Grafham D.V., Griffiths C., Griffiths M.N.D., Gwilliam R., Hall R.E., Hammond S., Harley J.L., Heath P.D., Ho S., Holden J.L., Howden P.J., Huckle E., Hunt A.R., Hunt S.E., Jekosch K., Johnson C.M., Johnson D., Kay M.P., Kimberley A.M., King A., Knights A., Laird G.K., Lawlor S., Lehvaeslaiho M.H., Leversha M.A., Lloyd C., Lloyd D.M., Lovell J.D., Marsh V.L., Martin S.L., McConnachie L.J., McLay K., McMurray A.A., Milne S.A., Mistry D., Moore M.J.F., Mullikin J.C., Nickerson T., Oliver K., Parker A., Patel R., Pearce T.A.V., Peck A.I., Phillimore B.J.C.T., Prathalingam S.R., Plumb R.W., Ramsay H., Rice C.M., Ross M.T., Scott C.E., Sehra H.K., Shownkeen R., Sims S., Skuce C.D., Smith M.L., Soderlund C., Steward C.A., Sulston J.E., Swann R.M., Sycamore N., Taylor R., Tee L., Thomas D.W., Thorpe A., Tracey A., Tromans A.C., Vaudin M., Wall M., Wallis J.M., Whitehead S.L., Whittaker P., Willey D.L., Williams L., Williams S.A., Wilming L., Wray P.W., Hubbard T., Durbin R.M., Bentley D.R., Beck S., Rogers J.
Nature 414:865-871(2001) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[5]Mural R.J., Istrail S., Sutton G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. expand/collapse author list , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C.
Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA], VARIANT ARG-258.
[6]"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 5).
Tissue: Blood.
[7]"The crystal structure of the second Z-DNA binding domain of human DAI (ZBP1) in complex with Z-DNA reveals an unusual binding mode to Z-DNA."
Ha S.C., Kim D., Hwang H.Y., Rich A., Kim Y.G., Kim K.K.
Proc. Natl. Acad. Sci. U.S.A. 105:20671-20676(2008) [PubMed] [Europe PMC] [Abstract]
Cited for: X-RAY CRYSTALLOGRAPHY (1.45 ANGSTROMS) OF 103-166, DNA-BINDING.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
AJ300575 mRNA. Translation: CAC18810.1.
CT002036 mRNA. No translation available.
AK122761 mRNA. Translation: BAG53713.1.
AL035541 Genomic DNA. Translation: CAI23107.1.
AL035541 Genomic DNA. Translation: CAM28299.1.
CH471077 Genomic DNA. Translation: EAW75511.1.
BC028218 mRNA. Translation: AAH28218.1.
CCDSCCDS13461.1. [Q9H171-1]
CCDS54477.1. [Q9H171-7]
CCDS54478.1. [Q9H171-6]
RefSeqNP_001153889.1. NM_001160417.1.
NP_001153890.1. NM_001160418.1. [Q9H171-7]
NP_001153891.1. NM_001160419.2. [Q9H171-6]
NP_110403.2. NM_030776.2. [Q9H171-1]
UniGeneHs.302123.

3D structure databases

PDBe
RCSB-PDB
PDBj
EntryMethodResolution (Å)ChainPositionsPDBsum
2L4MNMR-A103-166[»]
2LNBNMR-A6-74[»]
3EYIX-ray1.45A/B103-166[»]
4KA4X-ray2.60A/B/D/E96-165[»]
ProteinModelPortalQ9H171.
SMRQ9H171. Positions 6-74, 103-165.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid123349. 10 interactions.
DIPDIP-44121N.
IntActQ9H171. 6 interactions.
MINTMINT-6167522.
STRING9606.ENSP00000360215.

PTM databases

PhosphoSiteQ9H171.

Polymorphism databases

DMDM71153189.

Proteomic databases

PaxDbQ9H171.
PRIDEQ9H171.

Protocols and materials databases

DNASU81030.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000340462; ENSP00000344954; ENSG00000124256. [Q9H171-4]
ENST00000343535; ENSP00000340584; ENSG00000124256. [Q9H171-2]
ENST00000371173; ENSP00000360215; ENSG00000124256. [Q9H171-1]
ENST00000395822; ENSP00000379167; ENSG00000124256. [Q9H171-7]
ENST00000541799; ENSP00000440552; ENSG00000124256. [Q9H171-6]
GeneID81030.
KEGGhsa:81030.
UCSCuc002xyo.3. human. [Q9H171-1]

Organism-specific databases

CTD81030.
GeneCardsGC20M056178.
H-InvDBHIX0203057.
HGNCHGNC:16176. ZBP1.
HPAHPA041256.
MIM606750. gene.
neXtProtNX_Q9H171.
PharmGKBPA38094.
GenAtlasSearch...

Phylogenomic databases

eggNOGNOG38978.
HOGENOMHOG000230878.
HOVERGENHBG036279.
KOK12965.
OMARLEYAYH.
OrthoDBEOG70S75W.
PhylomeDBQ9H171.
TreeFamTF337658.

Enzyme and pathway databases

ReactomeREACT_6900. Immune System.

Gene expression databases

BgeeQ9H171.
CleanExHS_ZBP1.
GenevestigatorQ9H171.

Family and domain databases

Gene3D1.10.10.10. 2 hits.
InterProIPR000607. dsRNA_A_deaminase.
IPR025735. RHIM_dom.
IPR011991. WHTH_DNA-bd_dom.
[Graphical view]
PfamPF12721. RHIM. 2 hits.
PF02295. z-alpha. 2 hits.
[Graphical view]
ProtoNetSearch...

Other

EvolutionaryTraceQ9H171.
GeneWikiZBP1.
GenomeRNAi81030.
NextBio71358.
PROQ9H171.
SOURCESearch...

Entry information

Entry nameZBP1_HUMAN
AccessionPrimary (citable) accession number: Q9H171
Secondary accession number(s): A2A2F7 expand/collapse secondary AC list , B3KVA1, F5GYT1, Q5JY39, Q9BYW4
Entry history
Integrated into UniProtKB/Swiss-Prot: November 25, 2002
Last sequence update: July 19, 2005
Last modified: July 9, 2014
This is version 120 of the entry and version 2 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 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 20

Human chromosome 20: entries, gene names and cross-references to MIM