UniProtKB - Q9UJM8 (HAOX1_HUMAN)
Protein
Hydroxyacid oxidase 1
Gene
HAO1
Organism
Homo sapiens (Human)
Status
Functioni
Has 2-hydroxyacid oxidase activity. Most active on the 2-carbon substrate glycolate, but is also active on 2-hydroxy fatty acids, with high activity towards 2-hydroxy palmitate and 2-hydroxy octanoate.1 Publication
Catalytic activityi
- EC:1.1.3.151 Publication
Cofactori
FMNPROSITE-ProRule annotation1 Publication
Kineticsi
- KM=141 µM for glycolate1 Publication
- KM=40 µM for 2-hydroxy octanoate1 Publication
- KM=2200 µM for glyoxylate1 Publication
: glycolate degradation Pathwayi
This protein is involved in step 1 of the subpathway that synthesizes 3-phospho-D-glycerate from glycolate.Proteins known to be involved in the 4 steps of the subpathway in this organism are:
- Hydroxyacid oxidase 1 (HAO1)
- no protein annotated in this organism
- no protein annotated in this organism
- no protein annotated in this organism
View all proteins of this organism that are known to be involved in the subpathway that synthesizes 3-phospho-D-glycerate from glycolate, the pathway glycolate degradation and in Organic acid metabolism.
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Binding sitei | 26 | Substrate | 1 | |
Binding sitei | 108 | FMNPROSITE-ProRule annotation3 Publications | 1 | |
Binding sitei | 130 | FMNPROSITE-ProRule annotation3 Publications | 1 | |
Binding sitei | 132 | FMNPROSITE-ProRule annotation3 Publications | 1 | |
Binding sitei | 132 | Substrate | 1 | |
Binding sitei | 158 | FMNPROSITE-ProRule annotation3 Publications | 1 | |
Binding sitei | 167 | Substrate | 1 | |
Binding sitei | 236 | FMNPROSITE-ProRule annotation3 Publications | 1 | |
Binding sitei | 258 | FMNPROSITE-ProRule annotation3 Publications | 1 | |
Active sitei | 260 | Proton acceptor | 1 | |
Binding sitei | 263 | Substrate | 1 | |
Binding sitei | 315 | FMNPROSITE-ProRule annotation3 Publications | 1 |
Regions
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Nucleotide bindingi | 291 – 315 | FMNPROSITE-ProRule annotationAdd BLAST | 25 | |
Nucleotide bindingi | 291 – 295 | FMN bindingPROSITE-ProRule annotation3 Publications | 5 |
GO - Molecular functioni
- (S)-2-hydroxy-acid oxidase activity Source: UniProtKB
- FMN binding Source: UniProtKB
- glycolate oxidase activity Source: UniProtKB
- long-chain-(S)-2-hydroxy-long-chain-acid oxidase activity Source: UniProtKB-EC
- 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
- cellular nitrogen compound metabolic process Source: Reactome
- fatty acid alpha-oxidation Source: UniProtKB
- glycolate catabolic process Source: UniProtKB
- protein targeting to peroxisome Source: Reactome
- response to oxidative stress Source: Ensembl
Keywordsi
Molecular function | Oxidoreductase |
Ligand | Flavoprotein, FMN |
Enzyme and pathway databases
BRENDAi | 1.1.3.15, 2681 |
PathwayCommonsi | Q9UJM8 |
Reactomei | R-HSA-389661, Glyoxylate metabolism and glycine degradation R-HSA-9033241, Peroxisomal protein import |
SABIO-RKi | Q9UJM8 |
UniPathwayi | UPA00864;UER00830 |
Names & Taxonomyi
Protein namesi | Recommended name: Hydroxyacid oxidase 1 (EC:1.1.3.15)Short name: HAOX1 Alternative name(s): Glycolate oxidase Short name: GOX |
Gene namesi | Name:HAO1 Synonyms:GOX1, HAOX1 |
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:4809, HAO1 |
MIMi | 605023, gene |
neXtProti | NX_Q9UJM8 |
VEuPathDBi | HostDB:ENSG00000101323.4 |
Subcellular locationi
Peroxisome
Cytosol
- cytosol Source: Reactome
Peroxisome
- peroxisomal matrix Source: Reactome
- peroxisome Source: UniProtKB
Keywords - Cellular componenti
PeroxisomePathology & Biotechi
Organism-specific databases
DisGeNETi | 54363 |
OpenTargetsi | ENSG00000101323 |
PharmGKBi | PA29185 |
Miscellaneous databases
Pharosi | Q9UJM8, Tchem |
Chemistry databases
ChEMBLi | CHEMBL4229 |
DrugBanki | DB07907, (2S)-2-HYDROXYOCTANOIC ACID DB07060, 3-(INDOL-3-YL) LACTATE DB03064, 3-Decyl-2,5-Dioxo-4-Hydroxy-3-Pyrroline DB04374, 4-Carboxy-5-(1-Pentyl)Hexylsulfanyl-1,2,3-Triazole DB06979, 5-(dodecylthio)-1H-1,2,3-triazole-4-carboxylic acid DB02279, Benzoylformic Acid DB03247, Flavin mononucleotide DB02164, N-sulfo-flavin mononucleotide DB03884, Phenylpyruvic acid |
Genetic variation databases
BioMutai | HAO1 |
DMDMi | 13124294 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000206318 | 1 – 370 | Hydroxyacid oxidase 1Add BLAST | 370 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 184 | N6-succinyllysineBy similarity | 1 | |
Modified residuei | 194 | PhosphoserineBy similarity | 1 | |
Modified residuei | 230 | PhosphoserineCombined sources | 1 |
Keywords - PTMi
PhosphoproteinProteomic databases
MassIVEi | Q9UJM8 |
PaxDbi | Q9UJM8 |
PeptideAtlasi | Q9UJM8 |
PRIDEi | Q9UJM8 |
ProteomicsDBi | 84633 |
PTM databases
iPTMneti | Q9UJM8 |
PhosphoSitePlusi | Q9UJM8 |
Expressioni
Tissue specificityi
Liver.
Gene expression databases
Bgeei | ENSG00000101323, Expressed in right lobe of liver and 27 other tissues |
ExpressionAtlasi | Q9UJM8, baseline and differential |
Genevisiblei | Q9UJM8, HS |
Organism-specific databases
HPAi | ENSG00000101323, Tissue enriched (liver) |
Interactioni
Protein-protein interaction databases
BioGRIDi | 119941, 4 interactors |
IntActi | Q9UJM8, 3 interactors |
STRINGi | 9606.ENSP00000368066 |
Chemistry databases
BindingDBi | Q9UJM8 |
Miscellaneous databases
RNActi | Q9UJM8, protein |
Structurei
Secondary structure
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details3D structure databases
SMRi | Q9UJM8 |
ModBasei | Search... |
PDBe-KBi | Search... |
Miscellaneous databases
EvolutionaryTracei | Q9UJM8 |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 1 – 365 | FMN hydroxy acid dehydrogenasePROSITE-ProRule annotationAdd BLAST | 365 |
Motif
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Motifi | 368 – 370 | 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_6_1_1 |
InParanoidi | Q9UJM8 |
OMAi | RIWFRPQ |
OrthoDBi | 879633at2759 |
PhylomeDBi | Q9UJM8 |
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 |
i Sequence
Sequence statusi: Complete.
Q9UJM8-1 [UniParc]FASTAAdd to basket
10 20 30 40 50
MLPRLICIND YEQHAKSVLP KSIYDYYRSG ANDEETLADN IAAFSRWKLY
60 70 80 90 100
PRMLRNVAET DLSTSVLGQR VSMPICVGAT AMQRMAHVDG ELATVRACQS
110 120 130 140 150
LGTGMMLSSW ATSSIEEVAE AGPEALRWLQ LYIYKDREVT KKLVRQAEKM
160 170 180 190 200
GYKAIFVTVD TPYLGNRLDD VRNRFKLPPQ LRMKNFETST LSFSPEENFG
210 220 230 240 250
DDSGLAAYVA KAIDPSISWE DIKWLRRLTS LPIVAKGILR GDDAREAVKH
260 270 280 290 300
GLNGILVSNH GARQLDGVPA TIDVLPEIVE AVEGKVEVFL DGGVRKGTDV
310 320 330 340 350
LKALALGAKA VFVGRPIVWG LAFQGEKGVQ DVLEILKEEF RLAMALSGCQ
360 370
NVKVIDKTLV RKNPLAVSKI
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AF244134 mRNA Translation: AAF63219.1 AF231916 mRNA Translation: AAF40199.1 AL121739 mRNA Translation: CAB57329.1 AB024079 mRNA Translation: BAA82872.1 AL021879 Genomic DNA No translation available. BC113665 mRNA Translation: AAI13666.1 BC113667 mRNA Translation: AAI13668.1 |
CCDSi | CCDS13100.1 |
RefSeqi | NP_060015.1, NM_017545.2 |
Genome annotation databases
Ensembli | ENST00000378789; ENSP00000368066; ENSG00000101323 |
GeneIDi | 54363 |
KEGGi | hsa:54363 |
UCSCi | uc002wmw.2, human |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AF244134 mRNA Translation: AAF63219.1 AF231916 mRNA Translation: AAF40199.1 AL121739 mRNA Translation: CAB57329.1 AB024079 mRNA Translation: BAA82872.1 AL021879 Genomic DNA No translation available. BC113665 mRNA Translation: AAI13666.1 BC113667 mRNA Translation: AAI13668.1 |
CCDSi | CCDS13100.1 |
RefSeqi | NP_060015.1, NM_017545.2 |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
2NZL | X-ray | 1.35 | A | 1-370 | [»] | |
2RDT | X-ray | 1.95 | A | 1-370 | [»] | |
2RDU | X-ray | 1.65 | A | 1-370 | [»] | |
2RDW | X-ray | 1.95 | A | 1-370 | [»] | |
2W0U | X-ray | 2.84 | A/B/C/D | 1-370 | [»] | |
5QIB | X-ray | 1.48 | A | 1-368 | [»] | |
5QIC | X-ray | 1.34 | A | 1-368 | [»] | |
5QID | X-ray | 1.45 | A | 1-368 | [»] | |
5QIE | X-ray | 1.34 | A | 1-368 | [»] | |
5QIF | X-ray | 1.20 | A | 1-368 | [»] | |
5QIG | X-ray | 1.42 | A | 1-368 | [»] | |
5QIH | X-ray | 1.33 | A | 1-368 | [»] | |
6GMB | X-ray | 1.35 | A | 1-362 | [»] | |
6GMC | X-ray | 1.20 | A | 1-362 | [»] | |
SMRi | Q9UJM8 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 119941, 4 interactors |
IntActi | Q9UJM8, 3 interactors |
STRINGi | 9606.ENSP00000368066 |
Chemistry databases
BindingDBi | Q9UJM8 |
ChEMBLi | CHEMBL4229 |
DrugBanki | DB07907, (2S)-2-HYDROXYOCTANOIC ACID DB07060, 3-(INDOL-3-YL) LACTATE DB03064, 3-Decyl-2,5-Dioxo-4-Hydroxy-3-Pyrroline DB04374, 4-Carboxy-5-(1-Pentyl)Hexylsulfanyl-1,2,3-Triazole DB06979, 5-(dodecylthio)-1H-1,2,3-triazole-4-carboxylic acid DB02279, Benzoylformic Acid DB03247, Flavin mononucleotide DB02164, N-sulfo-flavin mononucleotide DB03884, Phenylpyruvic acid |
PTM databases
iPTMneti | Q9UJM8 |
PhosphoSitePlusi | Q9UJM8 |
Genetic variation databases
BioMutai | HAO1 |
DMDMi | 13124294 |
Proteomic databases
MassIVEi | Q9UJM8 |
PaxDbi | Q9UJM8 |
PeptideAtlasi | Q9UJM8 |
PRIDEi | Q9UJM8 |
ProteomicsDBi | 84633 |
Protocols and materials databases
Antibodypediai | 8448, 380 antibodies |
DNASUi | 54363 |
Genome annotation databases
Ensembli | ENST00000378789; ENSP00000368066; ENSG00000101323 |
GeneIDi | 54363 |
KEGGi | hsa:54363 |
UCSCi | uc002wmw.2, human |
Organism-specific databases
CTDi | 54363 |
DisGeNETi | 54363 |
GeneCardsi | HAO1 |
HGNCi | HGNC:4809, HAO1 |
HPAi | ENSG00000101323, Tissue enriched (liver) |
MIMi | 605023, gene |
neXtProti | NX_Q9UJM8 |
OpenTargetsi | ENSG00000101323 |
PharmGKBi | PA29185 |
VEuPathDBi | HostDB:ENSG00000101323.4 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG0538, Eukaryota |
GeneTreei | ENSGT00390000018717 |
HOGENOMi | CLU_020639_6_1_1 |
InParanoidi | Q9UJM8 |
OMAi | RIWFRPQ |
OrthoDBi | 879633at2759 |
PhylomeDBi | Q9UJM8 |
TreeFami | TF313363 |
Enzyme and pathway databases
UniPathwayi | UPA00864;UER00830 |
BRENDAi | 1.1.3.15, 2681 |
PathwayCommonsi | Q9UJM8 |
Reactomei | R-HSA-389661, Glyoxylate metabolism and glycine degradation R-HSA-9033241, Peroxisomal protein import |
SABIO-RKi | Q9UJM8 |
Miscellaneous databases
BioGRID-ORCSi | 54363, 1 hit in 870 CRISPR screens |
EvolutionaryTracei | Q9UJM8 |
GenomeRNAii | 54363 |
Pharosi | Q9UJM8, Tchem |
PROi | PR:Q9UJM8 |
RNActi | Q9UJM8, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000101323, Expressed in right lobe of liver and 27 other tissues |
ExpressionAtlasi | Q9UJM8, baseline and differential |
Genevisiblei | Q9UJM8, 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 | HAOX1_HUMAN | |
Accessioni | Q9UJM8Primary (citable) accession number: Q9UJM8 Secondary accession number(s): Q14CQ0, Q9UPZ0, Q9Y3I7 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | February 21, 2001 |
Last sequence update: | May 1, 2000 | |
Last modified: | February 10, 2021 | |
This is version 177 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
3D-structure, Reference proteomeDocuments
- Human chromosome 20
Human chromosome 20: entries, gene names and cross-references to MIM - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PATHWAY comments
Index of metabolic and biosynthesis pathways - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families