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Protein

3-hydroxyanthranilate 3,4-dioxygenase

Gene

HAAO

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

Functioni

Catalyzes the oxidative ring opening of 3-hydroxyanthranilate to 2-amino-3-carboxymuconate semialdehyde, which spontaneously cyclizes to quinolinate.UniRule annotation1 Publication

Catalytic activityi

3-hydroxyanthranilate + O2 = 2-amino-3-carboxymuconate semialdehyde.UniRule annotation1 Publication

Cofactori

Fe2+UniRule annotation1 Publication

Pathwayi: NAD(+) biosynthesis

This protein is involved in step 3 of the subpathway that synthesizes quinolinate from L-kynurenine.UniRule annotation
Proteins known to be involved in the 3 steps of the subpathway in this organism are:
  1. Kynurenine 3-monooxygenase (KMO), Kynurenine 3-monooxygenase (KMO), Kynurenine 3-monooxygenase (KMO), Kynurenine 3-monooxygenase (KMO)
  2. Kynureninase (KYNU), Kynureninase (KYNU), Kynureninase (KYNU)
  3. 3-hydroxyanthranilate 3,4-dioxygenase (HAAO)
This subpathway is part of the pathway NAD(+) biosynthesis, which is itself part of Cofactor biosynthesis.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes quinolinate from L-kynurenine, the pathway NAD(+) biosynthesis and in Cofactor biosynthesis.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei43DioxygenUniRule annotation1
Metal bindingi47Iron; catalyticUniRule annotation1
Metal bindingi53Iron; catalyticUniRule annotation1
Binding sitei53SubstrateUniRule annotation1
Metal bindingi91Iron; catalyticUniRule annotation1
Binding sitei95SubstrateUniRule annotation1
Binding sitei105SubstrateUniRule annotation1

GO - Molecular functioni

  • 3-hydroxyanthranilate 3,4-dioxygenase activity Source: UniProtKB
  • electron carrier activity Source: UniProtKB
  • ferrous iron binding Source: UniProtKB

GO - Biological processi

  • 'de novo' NAD biosynthetic process from tryptophan Source: GO_Central
  • anthranilate metabolic process Source: UniProtKB-HAMAP
  • neuron cellular homeostasis Source: UniProtKB
  • quinolinate biosynthetic process Source: UniProtKB
  • quinolinate metabolic process Source: GO_Central
  • response to cadmium ion Source: UniProtKB
  • response to zinc ion Source: UniProtKB
  • tryptophan catabolic process Source: Reactome
Complete GO annotation...

Keywords - Molecular functioni

Dioxygenase, Oxidoreductase

Keywords - Biological processi

Pyridine nucleotide biosynthesis

Keywords - Ligandi

Iron, Metal-binding

Enzyme and pathway databases

BioCyciMetaCyc:HS08749-MONOMER.
ZFISH:HS08749-MONOMER.
ReactomeiR-HSA-71240. Tryptophan catabolism.
UniPathwayiUPA00253; UER00330.

Names & Taxonomyi

Protein namesi
Recommended name:
3-hydroxyanthranilate 3,4-dioxygenaseUniRule annotation (EC:1.13.11.6UniRule annotation)
Alternative name(s):
3-hydroxyanthranilate oxygenaseUniRule annotation
Short name:
3-HAOUniRule annotation
3-hydroxyanthranilic acid dioxygenaseUniRule annotation
Short name:
HADUniRule annotation
Gene namesi
Name:HAAOUniRule annotation
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 2

Organism-specific databases

HGNCiHGNC:4796. HAAO.

Subcellular locationi

GO - Cellular componenti

  • cytosol Source: UniProtKB
  • extracellular exosome Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm

Pathology & Biotechi

Organism-specific databases

DisGeNETi23498.
OpenTargetsiENSG00000162882.
PharmGKBiPA29171.

Chemistry databases

ChEMBLiCHEMBL3108657.

Polymorphism and mutation databases

BioMutaiHAAO.
DMDMi308153402.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000643721 – 2863-hydroxyanthranilate 3,4-dioxygenaseAdd BLAST286

Proteomic databases

EPDiP46952.
MaxQBiP46952.
PaxDbiP46952.
PeptideAtlasiP46952.
PRIDEiP46952.

PTM databases

iPTMnetiP46952.
PhosphoSitePlusiP46952.

Expressioni

Gene expression databases

BgeeiENSG00000162882.
CleanExiHS_HAAO.
ExpressionAtlasiP46952. baseline and differential.
GenevisibleiP46952. HS.

Organism-specific databases

HPAiHPA036394.
HPA042024.

Interactioni

Subunit structurei

Monomer.UniRule annotation1 Publication

Binary interactionsi

WithEntry#Exp.IntActNotes
GAD1Q8IVA83EBI-743215,EBI-10209663
GAD1Q992596EBI-743215,EBI-743184
POT1Q9NUX52EBI-743215,EBI-752420

Protein-protein interaction databases

BioGridi117047. 5 interactors.
IntActiP46952. 5 interactors.
MINTiMINT-1468803.
STRINGi9606.ENSP00000294973.

Chemistry databases

BindingDBiP46952.

Structurei

Secondary structure

1286
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi4 – 6Combined sources3
Helixi7 – 13Combined sources7
Helixi15 – 17Combined sources3
Turni20 – 22Combined sources3
Beta strandi24 – 37Combined sources14
Beta strandi46 – 48Combined sources3
Beta strandi53 – 60Combined sources8
Beta strandi62 – 68Combined sources7
Beta strandi71 – 77Combined sources7
Beta strandi81 – 85Combined sources5
Beta strandi91 – 95Combined sources5
Beta strandi100 – 106Combined sources7
Beta strandi114 – 120Combined sources7
Beta strandi123 – 132Combined sources10
Helixi137 – 149Combined sources13
Helixi152 – 156Combined sources5
Helixi161 – 163Combined sources3
Helixi183 – 189Combined sources7
Helixi191 – 195Combined sources5
Beta strandi200 – 203Combined sources4
Beta strandi207 – 214Combined sources8
Beta strandi216 – 221Combined sources6
Beta strandi228 – 235Combined sources8
Beta strandi237 – 241Combined sources5
Beta strandi244 – 248Combined sources5
Beta strandi252 – 256Combined sources5
Beta strandi262 – 266Combined sources5
Beta strandi271 – 277Combined sources7
Helixi279 – 281Combined sources3

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2QNKX-ray1.60A2-286[»]
ProteinModelPortaliP46952.
SMRiP46952.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP46952.

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni1 – 160Domain A (catalytic)UniRule annotationAdd BLAST160
Regioni161 – 177LinkerUniRule annotationAdd BLAST17
Regioni178 – 286Domain BUniRule annotationAdd BLAST109

Sequence similaritiesi

Belongs to the 3-HAO family.UniRule annotation

Phylogenomic databases

eggNOGiKOG3995. Eukaryota.
ENOG4111GH8. LUCA.
GeneTreeiENSGT00390000013008.
HOGENOMiHOG000218448.
HOVERGENiHBG000018.
InParanoidiP46952.
KOiK00452.
OMAiCKDLGTQ.
OrthoDBiEOG091G0IJ1.
PhylomeDBiP46952.
TreeFamiTF300246.

Family and domain databases

Gene3Di2.60.120.10. 2 hits.
HAMAPiMF_00825. 3_HAO. 1 hit.
InterProiIPR010329. 3hydroanth_dOase.
IPR016700. 3hydroanth_dOase_met.
IPR014710. RmlC-like_jellyroll.
IPR011051. RmlC_Cupin.
[Graphical view]
PANTHERiPTHR15497. PTHR15497. 1 hit.
PfamiPF06052. 3-HAO. 1 hit.
[Graphical view]
PIRSFiPIRSF017681. 3hydroanth_dOase_animal. 1 hit.
SUPFAMiSSF51182. SSF51182. 2 hits.
TIGRFAMsiTIGR03037. anthran_nbaC. 1 hit.

Sequences (2)i

Sequence statusi: Complete.

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

Isoform 1 (identifier: P46952-1) [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
MERRLGVRAW VKENRGSFQP PVCNKLMHQE QLKVMFIGGP NTRKDYHIEE
60 70 80 90 100
GEEVFYQLEG DMVLRVLEQG KHRDVVIRQG EIFLLPARVP HSPQRFANTV
110 120 130 140 150
GLVVERRRLE TELDGLRYYV GDTMDVLFEK WFYCKDLGTQ LAPIIQEFFS
160 170 180 190 200
SEQYRTGKPI PDQLLKEPPF PLSTRSIMEP MSLDAWLDSH HRELQAGTPL
210 220 230 240 250
SLFGDTYETQ VIAYGQGSSE GLRQNVDVWL WQLEGSSVVT MGGRRLSLAP
260 270 280
DDSLLVLAGT SYAWERTQGS VALSVTQDPA CKKPLG
Length:286
Mass (Da):32,556
Last modified:October 5, 2010 - v2
Checksum:i4DA6AC20FC6EC885
GO
Isoform 2 (identifier: P46952-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     148-286: Missing.

Show »
Length:147
Mass (Da):17,275
Checksum:i3ABF821D6C31CE9E
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_02150737I → V.3 PublicationsCorresponds to variant rs3816183dbSNPEnsembl.1
Natural variantiVAR_03047042T → S.Corresponds to variant rs3816182dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_036239148 – 286Missing in isoform 2. 2 PublicationsAdd BLAST139

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Z29481 mRNA. Translation: CAA82618.1.
CR457063 mRNA. Translation: CAG33344.1.
CR624693 mRNA. No translation available.
AK295693 mRNA. Translation: BAG58544.1.
AC098824 Genomic DNA. Translation: AAY14701.1.
CH471053 Genomic DNA. Translation: EAX00309.1.
BC029510 mRNA. Translation: AAH29510.1.
CCDSiCCDS33187.1. [P46952-1]
PIRiA54070.
RefSeqiNP_036337.2. NM_012205.2. [P46952-1]
UniGeneiHs.368805.

Genome annotation databases

EnsembliENST00000294973; ENSP00000294973; ENSG00000162882. [P46952-1]
GeneIDi23498.
KEGGihsa:23498.
UCSCiuc002rst.5. human. [P46952-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Z29481 mRNA. Translation: CAA82618.1.
CR457063 mRNA. Translation: CAG33344.1.
CR624693 mRNA. No translation available.
AK295693 mRNA. Translation: BAG58544.1.
AC098824 Genomic DNA. Translation: AAY14701.1.
CH471053 Genomic DNA. Translation: EAX00309.1.
BC029510 mRNA. Translation: AAH29510.1.
CCDSiCCDS33187.1. [P46952-1]
PIRiA54070.
RefSeqiNP_036337.2. NM_012205.2. [P46952-1]
UniGeneiHs.368805.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2QNKX-ray1.60A2-286[»]
ProteinModelPortaliP46952.
SMRiP46952.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi117047. 5 interactors.
IntActiP46952. 5 interactors.
MINTiMINT-1468803.
STRINGi9606.ENSP00000294973.

Chemistry databases

BindingDBiP46952.
ChEMBLiCHEMBL3108657.

PTM databases

iPTMnetiP46952.
PhosphoSitePlusiP46952.

Polymorphism and mutation databases

BioMutaiHAAO.
DMDMi308153402.

Proteomic databases

EPDiP46952.
MaxQBiP46952.
PaxDbiP46952.
PeptideAtlasiP46952.
PRIDEiP46952.

Protocols and materials databases

DNASUi23498.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000294973; ENSP00000294973; ENSG00000162882. [P46952-1]
GeneIDi23498.
KEGGihsa:23498.
UCSCiuc002rst.5. human. [P46952-1]

Organism-specific databases

CTDi23498.
DisGeNETi23498.
GeneCardsiHAAO.
HGNCiHGNC:4796. HAAO.
HPAiHPA036394.
HPA042024.
MIMi604521. gene.
neXtProtiNX_P46952.
OpenTargetsiENSG00000162882.
PharmGKBiPA29171.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3995. Eukaryota.
ENOG4111GH8. LUCA.
GeneTreeiENSGT00390000013008.
HOGENOMiHOG000218448.
HOVERGENiHBG000018.
InParanoidiP46952.
KOiK00452.
OMAiCKDLGTQ.
OrthoDBiEOG091G0IJ1.
PhylomeDBiP46952.
TreeFamiTF300246.

Enzyme and pathway databases

UniPathwayiUPA00253; UER00330.
BioCyciMetaCyc:HS08749-MONOMER.
ZFISH:HS08749-MONOMER.
ReactomeiR-HSA-71240. Tryptophan catabolism.

Miscellaneous databases

ChiTaRSiHAAO. human.
EvolutionaryTraceiP46952.
GenomeRNAii23498.
PROiP46952.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000162882.
CleanExiHS_HAAO.
ExpressionAtlasiP46952. baseline and differential.
GenevisibleiP46952. HS.

Family and domain databases

Gene3Di2.60.120.10. 2 hits.
HAMAPiMF_00825. 3_HAO. 1 hit.
InterProiIPR010329. 3hydroanth_dOase.
IPR016700. 3hydroanth_dOase_met.
IPR014710. RmlC-like_jellyroll.
IPR011051. RmlC_Cupin.
[Graphical view]
PANTHERiPTHR15497. PTHR15497. 1 hit.
PfamiPF06052. 3-HAO. 1 hit.
[Graphical view]
PIRSFiPIRSF017681. 3hydroanth_dOase_animal. 1 hit.
SUPFAMiSSF51182. SSF51182. 2 hits.
TIGRFAMsiTIGR03037. anthran_nbaC. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry namei3HAO_HUMAN
AccessioniPrimary (citable) accession number: P46952
Secondary accession number(s): A6NE56
, B4DIN2, Q53QZ7, Q8N6N9
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1995
Last sequence update: October 5, 2010
Last modified: November 30, 2016
This is version 150 of the entry and version 2 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 2
    Human chromosome 2: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. 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.