UniProtKB - Q9NYQ3 (HAOX2_HUMAN)
Protein
Hydroxyacid oxidase 2
Gene
HAO2
Organism
Homo sapiens (Human)
Status
Functioni
Catalyzes the oxidation of L-alpha-hydroxy acids as well as, more slowly, that of L-alpha-amino acids.
Catalytic activityi
- EC:1.1.3.15
Cofactori
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Binding sitei | 106 | FMNPROSITE-ProRule annotation | 1 | |
Binding sitei | 128 | FMNPROSITE-ProRule annotation | 1 | |
Binding sitei | 130 | SubstratePROSITE-ProRule annotation | 1 | |
Binding sitei | 156 | FMNPROSITE-ProRule annotation | 1 | |
Binding sitei | 165 | SubstratePROSITE-ProRule annotation | 1 | |
Binding sitei | 222 | FMNPROSITE-ProRule annotation | 1 | |
Active sitei | 246 | Proton acceptorPROSITE-ProRule annotation | 1 | |
Binding sitei | 249 | SubstratePROSITE-ProRule annotation | 1 | |
Binding sitei | 301 | FMNPROSITE-ProRule annotation | 1 |
Regions
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Nucleotide bindingi | 277 – 301 | FMNPROSITE-ProRule annotationAdd BLAST | 25 |
GO - Molecular functioni
- (S)-2-hydroxy-acid oxidase activity Source: UniProtKB
- FMN binding Source: InterPro
- long-chain-(S)-2-hydroxy-long-chain-acid oxidase activity Source: UniProtKB
- medium-chain-(S)-2-hydroxy-acid oxidase activity Source: UniProtKB-EC
- very-long-chain-(S)-2-hydroxy-acid oxidase activity Source: UniProtKB-EC
GO - Biological processi
- fatty acid catabolic process Source: Reactome
- fatty acid oxidation Source: UniProtKB
- protein localization Source: Reactome
Keywordsi
Molecular function | Oxidoreductase |
Ligand | Flavoprotein, FMN |
Enzyme and pathway databases
PathwayCommonsi | Q9NYQ3 |
Reactomei | R-HSA-390918, Peroxisomal lipid metabolism R-HSA-9033241, Peroxisomal protein import |
Names & Taxonomyi
Protein namesi | Recommended name: Hydroxyacid oxidase 2 (EC:1.1.3.15)Short name: HAOX2 Alternative name(s): (S)-2-hydroxy-acid oxidase, peroxisomal Cell growth-inhibiting gene 16 protein Long chain alpha-hydroxy acid oxidase Long-chain L-2-hydroxy acid oxidase |
Gene namesi | Name:HAO2 Synonyms:HAOX2 ORF Names:GIG16 |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:4810, HAO2 |
MIMi | 605176, gene |
neXtProti | NX_Q9NYQ3 |
VEuPathDBi | HostDB:ENSG00000116882.14 |
Subcellular locationi
Peroxisome
Cytosol
- cytosol Source: Reactome
Peroxisome
- peroxisomal matrix Source: Reactome
- peroxisome Source: UniProtKB
Keywords - Cellular componenti
PeroxisomePathology & Biotechi
Organism-specific databases
DisGeNETi | 51179 |
OpenTargetsi | ENSG00000116882 |
PharmGKBi | PA29186 |
Miscellaneous databases
Pharosi | Q9NYQ3, Tchem |
Chemistry databases
ChEMBLi | CHEMBL2169732 |
DrugBanki | DB03247, Flavin mononucleotide |
Genetic variation databases
BioMutai | HAO2 |
DMDMi | 13124287 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000206320 | 1 – 351 | Hydroxyacid oxidase 2Add BLAST | 351 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 178 | PhosphothreonineCombined sources | 1 |
Keywords - PTMi
PhosphoproteinProteomic databases
MassIVEi | Q9NYQ3 |
PaxDbi | Q9NYQ3 |
PeptideAtlasi | Q9NYQ3 |
PRIDEi | Q9NYQ3 |
ProteomicsDBi | 83262 [Q9NYQ3-1] |
PTM databases
iPTMneti | Q9NYQ3 |
PhosphoSitePlusi | Q9NYQ3 |
Expressioni
Tissue specificityi
Liver and kidney.
Gene expression databases
Bgeei | ENSG00000116882, Expressed in right lobe of liver and 108 other tissues |
ExpressionAtlasi | Q9NYQ3, baseline and differential |
Genevisiblei | Q9NYQ3, HS |
Organism-specific databases
HPAi | ENSG00000116882, Group enriched (kidney, liver) |
Interactioni
Subunit structurei
Homotetramer or homooctamer.
By similarityBinary interactionsi
Q9NYQ3
With | #Exp. | IntAct |
---|---|---|
SEC14L4 [Q9UDX3] | 3 | EBI-8803200,EBI-10320311 |
Protein-protein interaction databases
BioGRIDi | 119356, 26 interactors |
IntActi | Q9NYQ3, 26 interactors |
STRINGi | 9606.ENSP00000483507 |
Chemistry databases
BindingDBi | Q9NYQ3 |
Miscellaneous databases
RNActi | Q9NYQ3, protein |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 2 – 351 | FMN hydroxy acid dehydrogenasePROSITE-ProRule annotationAdd BLAST | 350 |
Motif
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Motifi | 349 – 351 | Microbody targeting signalSequence analysis | 3 |
Sequence similaritiesi
Belongs to the FMN-dependent alpha-hydroxy acid dehydrogenase family.PROSITE-ProRule annotation
Phylogenomic databases
eggNOGi | KOG0538, Eukaryota |
GeneTreei | ENSGT00390000018717 |
HOGENOMi | CLU_020639_0_0_1 |
InParanoidi | Q9NYQ3 |
OMAi | KAANCSA |
PhylomeDBi | Q9NYQ3 |
TreeFami | TF313363 |
Family and domain databases
CDDi | cd02809, alpha_hydroxyacid_oxid_FMN, 1 hit |
Gene3Di | 3.20.20.70, 1 hit |
InterProi | View protein in InterPro IPR013785, Aldolase_TIM IPR012133, Alpha-hydoxy_acid_DH_FMN IPR000262, FMN-dep_DH IPR037396, FMN_HAD IPR008259, FMN_hydac_DH_AS |
Pfami | View protein in Pfam PF01070, FMN_dh, 1 hit |
PIRSFi | PIRSF000138, Al-hdrx_acd_dh, 1 hit |
PROSITEi | View protein in PROSITE PS00557, FMN_HYDROXY_ACID_DH_1, 1 hit PS51349, FMN_HYDROXY_ACID_DH_2, 1 hit |
s (2+)i Sequence
Sequence statusi: Complete.
This entry describes 2 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 2 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All
Isoform 1 (identifier: Q9NYQ3-1) [UniParc]FASTAAdd to basket
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MSLVCLTDFQ AHAREQLSKS TRDFIEGGAD DSITRDDNIA AFKRIRLRPR
60 70 80 90 100
YLRDVSEVDT RTTIQGEEIS APICIAPTGF HCLVWPDGEM STARAAQAAG
110 120 130 140 150
ICYITSTFAS CSLEDIVIAA PEGLRWFQLY VHPDLQLNKQ LIQRVESLGF
160 170 180 190 200
KALVITLDTP VCGNRRHDIR NQLRRNLTLT DLQSPKKGNA IPYFQMTPIS
210 220 230 240 250
TSLCWNDLSW FQSITRLPII LKGILTKEDA ELAVKHNVQG IIVSNHGGRQ
260 270 280 290 300
LDEVLASIDA LTEVVAAVKG KIEVYLDGGV RTGNDVLKAL ALGAKCIFLG
310 320 330 340 350
RPILWGLACK GEHGVKEVLN ILTNEFHTSM ALTGCRSVAE INRNLVQFSR
L
Computationally mapped potential isoform sequencesi
There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basketQ5QP02 | Q5QP02_HUMAN | Hydroxyacid oxidase 2 | HAO2 | 186 | Annotation score: |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 80 | F → Y in AAF14000 (Ref. 2) Curated | 1 | |
Sequence conflicti | 292 – 294 | LGA → HED in AAF14000 (Ref. 2) Curated | 3 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_049087 | 15 | E → K. Corresponds to variant dbSNP:rs34638261Ensembl. | 1 | |
Natural variantiVAR_049088 | 221 | L → M. Corresponds to variant dbSNP:rs6661625Ensembl. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_055095 | 1 | M → MEDKMWSECEGPEM in isoform 2. 1 Publication | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AF231917 mRNA Translation: AAF40200.1 AF203975 mRNA Translation: AAF14000.1 AY513277 mRNA Translation: AAT08030.1 AK298289 mRNA Translation: BAG60549.1 AL139346 Genomic DNA No translation available. AL359553 Genomic DNA No translation available. CH471122 Genomic DNA Translation: EAW56698.1 CH471122 Genomic DNA Translation: EAW56699.1 BC020863 mRNA Translation: AAH20863.1 |
CCDSi | CCDS76196.1 [Q9NYQ3-2] CCDS901.1 [Q9NYQ3-1] |
RefSeqi | NP_001005783.2, NM_001005783.2 [Q9NYQ3-2] NP_057611.1, NM_016527.3 [Q9NYQ3-1] |
Genome annotation databases
Ensembli | ENST00000325945; ENSP00000316339; ENSG00000116882 [Q9NYQ3-1] ENST00000361035; ENSP00000354314; ENSG00000116882 [Q9NYQ3-2] ENST00000622548; ENSP00000483507; ENSG00000116882 [Q9NYQ3-1] |
GeneIDi | 51179 |
KEGGi | hsa:51179 |
UCSCi | uc001ehq.1, human [Q9NYQ3-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AF231917 mRNA Translation: AAF40200.1 AF203975 mRNA Translation: AAF14000.1 AY513277 mRNA Translation: AAT08030.1 AK298289 mRNA Translation: BAG60549.1 AL139346 Genomic DNA No translation available. AL359553 Genomic DNA No translation available. CH471122 Genomic DNA Translation: EAW56698.1 CH471122 Genomic DNA Translation: EAW56699.1 BC020863 mRNA Translation: AAH20863.1 |
CCDSi | CCDS76196.1 [Q9NYQ3-2] CCDS901.1 [Q9NYQ3-1] |
RefSeqi | NP_001005783.2, NM_001005783.2 [Q9NYQ3-2] NP_057611.1, NM_016527.3 [Q9NYQ3-1] |
3D structure databases
SMRi | Q9NYQ3 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 119356, 26 interactors |
IntActi | Q9NYQ3, 26 interactors |
STRINGi | 9606.ENSP00000483507 |
Chemistry databases
BindingDBi | Q9NYQ3 |
ChEMBLi | CHEMBL2169732 |
DrugBanki | DB03247, Flavin mononucleotide |
PTM databases
iPTMneti | Q9NYQ3 |
PhosphoSitePlusi | Q9NYQ3 |
Genetic variation databases
BioMutai | HAO2 |
DMDMi | 13124287 |
Proteomic databases
MassIVEi | Q9NYQ3 |
PaxDbi | Q9NYQ3 |
PeptideAtlasi | Q9NYQ3 |
PRIDEi | Q9NYQ3 |
ProteomicsDBi | 83262 [Q9NYQ3-1] |
Protocols and materials databases
Antibodypediai | 33905, 113 antibodies |
DNASUi | 51179 |
Genome annotation databases
Ensembli | ENST00000325945; ENSP00000316339; ENSG00000116882 [Q9NYQ3-1] ENST00000361035; ENSP00000354314; ENSG00000116882 [Q9NYQ3-2] ENST00000622548; ENSP00000483507; ENSG00000116882 [Q9NYQ3-1] |
GeneIDi | 51179 |
KEGGi | hsa:51179 |
UCSCi | uc001ehq.1, human [Q9NYQ3-1] |
Organism-specific databases
CTDi | 51179 |
DisGeNETi | 51179 |
GeneCardsi | HAO2 |
HGNCi | HGNC:4810, HAO2 |
HPAi | ENSG00000116882, Group enriched (kidney, liver) |
MIMi | 605176, gene |
neXtProti | NX_Q9NYQ3 |
OpenTargetsi | ENSG00000116882 |
PharmGKBi | PA29186 |
VEuPathDBi | HostDB:ENSG00000116882.14 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG0538, Eukaryota |
GeneTreei | ENSGT00390000018717 |
HOGENOMi | CLU_020639_0_0_1 |
InParanoidi | Q9NYQ3 |
OMAi | KAANCSA |
PhylomeDBi | Q9NYQ3 |
TreeFami | TF313363 |
Enzyme and pathway databases
PathwayCommonsi | Q9NYQ3 |
Reactomei | R-HSA-390918, Peroxisomal lipid metabolism R-HSA-9033241, Peroxisomal protein import |
Miscellaneous databases
BioGRID-ORCSi | 51179, 26 hits in 985 CRISPR screens |
ChiTaRSi | HAO2, human |
GenomeRNAii | 51179 |
Pharosi | Q9NYQ3, Tchem |
PROi | PR:Q9NYQ3 |
RNActi | Q9NYQ3, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000116882, Expressed in right lobe of liver and 108 other tissues |
ExpressionAtlasi | Q9NYQ3, baseline and differential |
Genevisiblei | Q9NYQ3, HS |
Family and domain databases
CDDi | cd02809, alpha_hydroxyacid_oxid_FMN, 1 hit |
Gene3Di | 3.20.20.70, 1 hit |
InterProi | View protein in InterPro IPR013785, Aldolase_TIM IPR012133, Alpha-hydoxy_acid_DH_FMN IPR000262, FMN-dep_DH IPR037396, FMN_HAD IPR008259, FMN_hydac_DH_AS |
Pfami | View protein in Pfam PF01070, FMN_dh, 1 hit |
PIRSFi | PIRSF000138, Al-hdrx_acd_dh, 1 hit |
PROSITEi | View protein in PROSITE PS00557, FMN_HYDROXY_ACID_DH_1, 1 hit PS51349, FMN_HYDROXY_ACID_DH_2, 1 hit |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | HAOX2_HUMAN | |
Accessioni | Q9NYQ3Primary (citable) accession number: Q9NYQ3 Secondary accession number(s): Q2TU86, Q5QP00, Q9UJS6 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | February 21, 2001 |
Last sequence update: | October 1, 2000 | |
Last modified: | April 7, 2021 | |
This is version 164 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | 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. |
Miscellaneousi
Keywords - Technical termi
Reference proteomeDocuments
- Human chromosome 1
Human chromosome 1: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - SIMILARITY comments
Index of protein domains and families