Z-DNA-binding protein 1 - Homo sapiens (Human)

Preferred order of sections

Names and origin

Protein names

Recommended name:
Z-DNA-binding protein 1

Alternative name(s):
Tumor stroma and activated macrophage protein DLM-1

Gene names

Name:	ZBP1
Synonyms:	C20orf183, DLM1

Organism

Homo sapiens (Human)

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

Protein attributes

Sequence length	429 AA.
Sequence status	Complete.
Protein existence	Evidence 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.

Ontologies

Keywords
Biological process	Immunity Innate immunity
Coding sequence diversity	Alternative splicing Polymorphism
Domain	Repeat
Ligand	DNA-binding
Technical term	3D-structure Complete proteome Reference proteome
Gene Ontology (GO)
Biological process	innate immune response Inferred from electronic annotation. Source: UniProtKB-KW
Cellular component	cytoplasm Inferred from direct assay. Source: MGI nucleus Inferred from direct assay. Source: MGI
Molecular function	RNA binding Inferred from electronic annotation. Source: InterPro double-stranded RNA adenosine deaminase activity Inferred from electronic annotation. Source: InterPro left-handed Z-DNA binding Non-traceable author statement Ref.1. Source: UniProtKB
Complete GO annotation...

Alternative products

This entry describes 5 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.

Sequence annotation (Features)

Feature key

Position(s)

Length

Description

Graphical view

Feature identifier

Molecule processing

Chain

1 – 429

429

Z-DNA-binding protein 1

PRO_0000066564

Regions

Repeat

6 – 72

67

DRADA 1

Repeat

103 – 168

66

DRADA 2

Motif

195 – 219

25

RIP homotypic interaction motif (RHIM) 1

Motif

253 – 277

25

RIP homotypic interaction motif (RHIM) 2

Natural variations

Alternative sequence

87 – 149

63

AERPQ…YRMKS → GNCHPGEAGLTLQGASWQWT STDLSLGSNLNSATWELTGF LSLCLGFFFWLMELTAGLLG RGC in isoform 5.

VSP_004079

Alternative sequence

87 – 109

23

Missing in isoform 4.

VSP_004078

Alternative sequence

150 – 429

280

Missing in isoform 5.

VSP_004080

Alternative sequence

365 – 429

65

GRRPA…WGGGI → VAAFTEVLNPSKSFMKFGIN FFQTPVNVDSLTSSHESQMF LTACRLVIFSRRFSTYFAQI HLRNHSVLHLCPHKIYLLKN KK in isoform 2.

VSP_004081

Alternative sequence

365 – 429

65

GRRPA…WGGGI → GRRPADTQSRSFDGQE in isoform 3.

VSP_004082

Natural variant

17

1

R → I.
Corresponds to variant rs35813125 [ dbSNP | Ensembl ].

VAR_057030

Natural variant

53

1

K → R.
Corresponds to variant rs35895307 [ dbSNP | Ensembl ].

VAR_061728

Natural variant

70

1

G → R.
Corresponds to variant rs34964609 [ dbSNP | Ensembl ].

VAR_057031

Natural variant

88

1

E → K. Ref.1
Corresponds to variant rs2073145 [ dbSNP | Ensembl ].

VAR_014316

Natural variant

154

1

D → H.
Corresponds to variant rs16981187 [ dbSNP | Ensembl ].

VAR_057032

Natural variant

166

1

R → H.
Corresponds to variant rs34478944 [ dbSNP | Ensembl ].

VAR_057033

Natural variant

332

1

A → V.
Corresponds to variant rs41275648 [ dbSNP | Ensembl ].

VAR_061729

Experimental info

Sequence conflict

258

1

Q → R in CAC18810. Ref.1

Secondary structure

1

.

429

Helix Strand Turn

Details...

Sequences

Sequence		Length	Mass (Da)
Isoform 1 [UniParc]. Last modified July 19, 2005. Version 2. Checksum: 2C9B195112822991 Show »	FASTA	429	46,343
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 »	FASTA	446	48,754
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 GEDAVAAFTE VLNPSKSFMK FGINFFQTPV NVDSLTSSHE SQMFLTACRL VIFSRRFSTY 430 440 FAQIHLRNHS VLHLCPHKIY LLKNKK « Hide
Isoform 3 [UniParc]. Checksum: 247472C6F85CEB36 Show »	FASTA	380	41,028
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 GEDAGRRPAD TQSRSFDGQE « Hide
Isoform 4 [UniParc]. Checksum: 709506D06777E8F2 Show »	FASTA	406	43,786
10 20 30 40 50 60 MAQAPADPGR EGHLEQRILQ VLTEAGSPVK LAQLVKECQA PKRELNQVLY RMKKELKVSL 70 80 90 100 110 120 TSPATWCLGG TDPEGEGPAE LALSSPEEDI YRFLKDNGPQ RALVIAQALG MRTAKDVNRD 130 140 150 160 170 180 LYRMKSRHLL DMDEQSKAWT IYRPEDSGRR AKSASIIYQH NPINMICQNG PNSWISIANS 190 200 210 220 230 240 EAIQIGHGNI ITRQTVSRED GSAGPRHLPS MAPGDSSTWG TLVDPWGPQD IHMEQSILRR 250 260 270 280 290 300 VQLGHSNEMR LHGVPSEGPA HIPPGSPPVS ATAAGPEASF EARIPSPGTH PEGEAAQRIH 310 320 330 340 350 360 MKSCFLEDAT IGNSNKMSIS PGVAGPGGVA GSGEGEPGED AGRRPADTQS RSHFPRDIGQ 370 380 390 400 PITPSHSKLT PKLETMTLGN RSHKAAEGSH YVDEASHEGS WWGGGI « Hide
Isoform 5 [UniParc]. Checksum: 1D948BB00599314B Show »	FASTA	149	15,985
10 20 30 40 50 60 MAQAPADPGR EGHLEQRILQ VLTEAGSPVK LAQLVKECQA PKRELNQVLY RMKKELKVSL 70 80 90 100 110 120 TSPATWCLGG TDPEGEGPAE LALSSPGNCH PGEAGLTLQG ASWQWTSTDL SLGSNLNSAT 130 140 WELTGFLSLC LGFFFWLMEL TAGLLGRGC « Hide

References

	« Hide 'large scale' referencesShow '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: 11842111] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], ALTERNATIVE SPLICING, VARIANT LYS-88. Tissue: Spleen.
[2]	"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. Rogers J. Nature 414:865-871(2001) [PubMed: 11780052] [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: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 5). Tissue: Blood.
[4]	"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: 19095800] [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.
AL035541 Genomic DNA. Translation: CAI23107.1.
BC028218 mRNA. Translation: AAH28218.1.

IPI

IPI00006512.
IPI00181608.
IPI00219702.
IPI00219704.
IPI00334653.

RefSeq

NP_001153889.1. NM_001160417.1.
NP_001153890.1. NM_001160418.1.
NP_110403.2. NM_030776.2.

UniGene

Hs.302123.

3D structure databases

PDBe
RCSB PDB
PDBj

Entry	Method	Resolution (Å)	Chain	Positions	PDBsum
2L4M	NMR	-	A	103-166	[»]
3EYI	X-ray	1.45	A/B	103-166	[»]

ProteinModelPortal

Q9H171.

SMR

Q9H171. Positions 13-69, 103-166.

ModBase

Search...

Protein-protein interaction databases

DIP

DIP-44121N.

MINT

MINT-6167522.

STRING

Q9H171.

Polymorphism databases

DMDM

71153189.

Proteomic databases

PRIDE

Q9H171.

Protocols and materials databases

StructuralBiologyKnowledgebase

Search...

Genome annotation databases

Ensembl

ENST00000371173; ENSP00000360215; ENSG00000124256.

GeneID

81030.

KEGG

hsa:81030.

UCSC

uc002xyo.1. human.

Organism-specific databases

CTD

81030.

GeneCards

GC20M056178.

H-InvDB

HIX0203057.

HGNC

HGNC:16176. ZBP1.

MIM

606750. gene.

neXtProt

NX_Q9H171.

GenAtlas

Search...

Phylogenomic databases

GeneTree

ENSGT00390000002234.

HOVERGEN

HBG036279.

OMA

IRSCFLE.

OrthoDB

EOG4QZ7KZ.

Gene expression databases

ArrayExpress

Q9H171.

Bgee

Q9H171.

CleanEx

HS_ZBP1.

Genevestigator

Q9H171.

GermOnline

ENSG00000124256. Homo sapiens.

Family and domain databases

InterPro

IPR000607. dsRNA_A_deaminase.
IPR011991. WHTH_trsnscrt_rep_DNA-bd.
[Graphical view]

Gene3D

G3DSA:1.10.10.10. Wing_hlx_DNA_bd. 2 hits.

KO

K12965.

Pfam

PF02295. z-alpha. 2 hits.
[Graphical view]

PROSITE

PS50139. DRADA_REPEAT. False negative.
[Graphical view]

ProtoNet

Search...

Other

NextBio

71358.

SOURCE

Search...

Entry information

Entry name

ZBP1_HUMAN

Accession

Primary (citable) accession number: Q9H171
Secondary accession number(s): Q5JY39, Q9BYW4

Entry history

Integrated into UniProtKB/Swiss-Prot:	November 25, 2002
Last sequence update:	July 19, 2005
Last modified:	January 25, 2012
This is version 93 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any 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

Human chromosome 20

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

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

MIM cross-references

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

PDB cross-references

Index of Protein Data Bank (PDB) cross-references

SIMILARITY comments

Index of protein domains and families