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Protein

Hydroxyacid oxidase 1

Gene

HAO1

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

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

(S)-2-hydroxy acid + O2 = 2-oxo acid + H2O2.1 Publication

Cofactori

FMNPROSITE-ProRule annotation1 Publication

Kineticsi

  1. KM=141 µM for glycolate1 Publication
  2. KM=40 µM for 2-hydroxy octanoate1 Publication
  3. KM=2200 µM for glyoxylate1 Publication

    Pathwayi: glycolate degradation

    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:
    1. Hydroxyacid oxidase 1 (HAO1)
    2. no protein annotated in this organism
    3. no protein annotated in this organism
    4. no protein annotated in this organism
    This subpathway is part of the pathway glycolate degradation, which is itself part of Organic acid metabolism.
    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 keyPosition(s)DescriptionActionsGraphical viewLength
    Binding sitei26Substrate1
    Binding sitei108FMNPROSITE-ProRule annotation3 Publications1
    Binding sitei130FMNPROSITE-ProRule annotation3 Publications1
    Binding sitei132FMNPROSITE-ProRule annotation3 Publications1
    Binding sitei132Substrate1
    Binding sitei158FMNPROSITE-ProRule annotation3 Publications1
    Binding sitei167Substrate1
    Binding sitei236FMNPROSITE-ProRule annotation3 Publications1
    Binding sitei258FMNPROSITE-ProRule annotation3 Publications1
    Active sitei260Proton acceptor1
    Binding sitei263Substrate1
    Binding sitei315FMNPROSITE-ProRule annotation3 Publications1

    Regions

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Nucleotide bindingi291 – 315FMNPROSITE-ProRule annotationAdd BLAST25
    Nucleotide bindingi291 – 295FMN bindingPROSITE-ProRule annotation3 Publications5

    GO - Molecular functioni

    GO - Biological processi

    Keywordsi

    Molecular functionOxidoreductase
    LigandFlavoprotein, FMN

    Enzyme and pathway databases

    BRENDAi1.1.3.15 2681
    ReactomeiR-HSA-389661 Glyoxylate metabolism and glycine degradation
    R-HSA-9033241 Peroxisomal protein import
    SABIO-RKiQ9UJM8
    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
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    Proteomesi
    • UP000005640 Componenti: Chromosome 20

    Organism-specific databases

    EuPathDBiHostDB:ENSG00000101323.4
    HGNCiHGNC:4809 HAO1
    MIMi605023 gene
    neXtProtiNX_Q9UJM8

    Subcellular locationi

    Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

    Keywords - Cellular componenti

    Peroxisome

    Pathology & Biotechi

    Organism-specific databases

    DisGeNETi54363
    OpenTargetsiENSG00000101323
    PharmGKBiPA29185

    Chemistry databases

    ChEMBLiCHEMBL4229
    DrugBankiDB07907 (2S)-2-HYDROXYOCTANOIC ACID
    DB07060 3-(INDOL-3-YL) LACTATE
    DB03884 3-Phenylpyruvic Acid
    DB02279 Benzoylformic Acid
    DB02164 N-Sulfo-Flavin Mononucleotide
    DB03247 Riboflavin Monophosphate

    Polymorphism and mutation databases

    BioMutaiHAO1
    DMDMi13124294

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    ChainiPRO_00002063181 – 370Hydroxyacid oxidase 1Add BLAST370

    Amino acid modifications

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Modified residuei184N6-succinyllysineBy similarity1
    Modified residuei194PhosphoserineBy similarity1
    Modified residuei230PhosphoserineCombined sources1

    Keywords - PTMi

    Phosphoprotein

    Proteomic databases

    EPDiQ9UJM8
    PaxDbiQ9UJM8
    PeptideAtlasiQ9UJM8
    PRIDEiQ9UJM8
    ProteomicsDBi84633

    PTM databases

    iPTMnetiQ9UJM8
    PhosphoSitePlusiQ9UJM8

    Expressioni

    Tissue specificityi

    Liver.

    Gene expression databases

    BgeeiENSG00000101323 Expressed in 15 organ(s), highest expression level in right lobe of liver
    CleanExiHS_HAO1
    ExpressionAtlasiQ9UJM8 baseline and differential
    GenevisibleiQ9UJM8 HS

    Organism-specific databases

    HPAiHPA049552

    Interactioni

    GO - Molecular functioni

    Protein-protein interaction databases

    BioGridi119941, 4 interactors
    IntActiQ9UJM8, 1 interactor
    STRINGi9606.ENSP00000368066

    Chemistry databases

    BindingDBiQ9UJM8

    Structurei

    Secondary structure

    1370
    Legend: HelixTurnBeta strandPDB Structure known for this area
    Show more details

    3D structure databases

    ProteinModelPortaliQ9UJM8
    SMRiQ9UJM8
    ModBaseiSearch...
    MobiDBiSearch...

    Miscellaneous databases

    EvolutionaryTraceiQ9UJM8

    Family & Domainsi

    Domains and Repeats

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Domaini1 – 365FMN hydroxy acid dehydrogenasePROSITE-ProRule annotationAdd BLAST365

    Motif

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Motifi368 – 370Microbody targeting signalSequence analysis3

    Sequence similaritiesi

    Belongs to the FMN-dependent alpha-hydroxy acid dehydrogenase family.PROSITE-ProRule annotation

    Phylogenomic databases

    eggNOGiKOG0538 Eukaryota
    COG1304 LUCA
    GeneTreeiENSGT00390000018717
    HOGENOMiHOG000217463
    HOVERGENiHBG051881
    InParanoidiQ9UJM8
    KOiK11517
    OMAiKSVYDYY
    OrthoDBiEOG091G0ADA
    PhylomeDBiQ9UJM8
    TreeFamiTF313363

    Family and domain databases

    CDDicd02809 alpha_hydroxyacid_oxid_FMN, 1 hit
    Gene3Di3.20.20.70, 1 hit
    InterProiView protein in InterPro
    IPR013785 Aldolase_TIM
    IPR012133 Alpha-hydoxy_acid_DH_FMN
    IPR000262 FMN-dep_DH
    IPR037396 FMN_HAD
    IPR008259 FMN_hydac_DH_AS
    PfamiView protein in Pfam
    PF01070 FMN_dh, 1 hit
    PIRSFiPIRSF000138 Al-hdrx_acd_dh, 1 hit
    PROSITEiView protein in PROSITE
    PS00557 FMN_HYDROXY_ACID_DH_1, 1 hit
    PS51349 FMN_HYDROXY_ACID_DH_2, 1 hit

    Sequencei

    Sequence statusi: Complete.

    Q9UJM8-1 [UniParc]FASTAAdd to basket
    « Hide
            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
    Length:370
    Mass (Da):40,924
    Last modified:May 1, 2000 - v1
    Checksum:iC683C6F7CB5FD323
    GO

    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
    CCDSiCCDS13100.1
    RefSeqiNP_060015.1, NM_017545.2
    UniGeneiHs.193640

    Genome annotation databases

    EnsembliENST00000378789; ENSP00000368066; ENSG00000101323
    GeneIDi54363
    KEGGihsa:54363
    UCSCiuc002wmw.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
    CCDSiCCDS13100.1
    RefSeqiNP_060015.1, NM_017545.2
    UniGeneiHs.193640

    3D structure databases

    Select the link destinations:
    PDBei
    RCSB PDBi
    PDBji
    Links Updated
    PDB entryMethodResolution (Å)ChainPositionsPDBsum
    2NZLX-ray1.35A1-370[»]
    2RDTX-ray1.95A1-370[»]
    2RDUX-ray1.65A1-370[»]
    2RDWX-ray1.95A1-370[»]
    2W0UX-ray2.84A/B/C/D1-370[»]
    5QIBX-ray1.48A1-368[»]
    5QICX-ray1.34A1-368[»]
    5QIDX-ray1.45A1-368[»]
    5QIEX-ray1.34A1-368[»]
    5QIFX-ray1.20A1-368[»]
    5QIGX-ray1.42A1-368[»]
    5QIHX-ray1.33A1-368[»]
    6GMBX-ray1.35A1-362[»]
    6GMCX-ray1.20A1-362[»]
    ProteinModelPortaliQ9UJM8
    SMRiQ9UJM8
    ModBaseiSearch...
    MobiDBiSearch...

    Protein-protein interaction databases

    BioGridi119941, 4 interactors
    IntActiQ9UJM8, 1 interactor
    STRINGi9606.ENSP00000368066

    Chemistry databases

    BindingDBiQ9UJM8
    ChEMBLiCHEMBL4229
    DrugBankiDB07907 (2S)-2-HYDROXYOCTANOIC ACID
    DB07060 3-(INDOL-3-YL) LACTATE
    DB03884 3-Phenylpyruvic Acid
    DB02279 Benzoylformic Acid
    DB02164 N-Sulfo-Flavin Mononucleotide
    DB03247 Riboflavin Monophosphate

    PTM databases

    iPTMnetiQ9UJM8
    PhosphoSitePlusiQ9UJM8

    Polymorphism and mutation databases

    BioMutaiHAO1
    DMDMi13124294

    Proteomic databases

    EPDiQ9UJM8
    PaxDbiQ9UJM8
    PeptideAtlasiQ9UJM8
    PRIDEiQ9UJM8
    ProteomicsDBi84633

    Protocols and materials databases

    DNASUi54363
    Structural Biology KnowledgebaseSearch...

    Genome annotation databases

    EnsembliENST00000378789; ENSP00000368066; ENSG00000101323
    GeneIDi54363
    KEGGihsa:54363
    UCSCiuc002wmw.2 human

    Organism-specific databases

    CTDi54363
    DisGeNETi54363
    EuPathDBiHostDB:ENSG00000101323.4
    GeneCardsiHAO1
    HGNCiHGNC:4809 HAO1
    HPAiHPA049552
    MIMi605023 gene
    neXtProtiNX_Q9UJM8
    OpenTargetsiENSG00000101323
    PharmGKBiPA29185
    GenAtlasiSearch...

    Phylogenomic databases

    eggNOGiKOG0538 Eukaryota
    COG1304 LUCA
    GeneTreeiENSGT00390000018717
    HOGENOMiHOG000217463
    HOVERGENiHBG051881
    InParanoidiQ9UJM8
    KOiK11517
    OMAiKSVYDYY
    OrthoDBiEOG091G0ADA
    PhylomeDBiQ9UJM8
    TreeFamiTF313363

    Enzyme and pathway databases

    UniPathwayi
    UPA00864;UER00830

    BRENDAi1.1.3.15 2681
    ReactomeiR-HSA-389661 Glyoxylate metabolism and glycine degradation
    R-HSA-9033241 Peroxisomal protein import
    SABIO-RKiQ9UJM8

    Miscellaneous databases

    EvolutionaryTraceiQ9UJM8
    GenomeRNAii54363
    PROiPR:Q9UJM8
    SOURCEiSearch...

    Gene expression databases

    BgeeiENSG00000101323 Expressed in 15 organ(s), highest expression level in right lobe of liver
    CleanExiHS_HAO1
    ExpressionAtlasiQ9UJM8 baseline and differential
    GenevisibleiQ9UJM8 HS

    Family and domain databases

    CDDicd02809 alpha_hydroxyacid_oxid_FMN, 1 hit
    Gene3Di3.20.20.70, 1 hit
    InterProiView protein in InterPro
    IPR013785 Aldolase_TIM
    IPR012133 Alpha-hydoxy_acid_DH_FMN
    IPR000262 FMN-dep_DH
    IPR037396 FMN_HAD
    IPR008259 FMN_hydac_DH_AS
    PfamiView protein in Pfam
    PF01070 FMN_dh, 1 hit
    PIRSFiPIRSF000138 Al-hdrx_acd_dh, 1 hit
    PROSITEiView protein in PROSITE
    PS00557 FMN_HYDROXY_ACID_DH_1, 1 hit
    PS51349 FMN_HYDROXY_ACID_DH_2, 1 hit
    ProtoNetiSearch...

    Entry informationi

    Entry nameiHAOX1_HUMAN
    AccessioniPrimary (citable) accession number: Q9UJM8
    Secondary accession number(s): Q14CQ0, Q9UPZ0, Q9Y3I7
    Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 21, 2001
    Last sequence update: May 1, 2000
    Last modified: November 7, 2018
    This is version 161 of the entry and version 1 of the sequence. See 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. SIMILARITY comments
      Index of protein domains and families
    2. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    3. PATHWAY comments
      Index of metabolic and biosynthesis pathways
    4. Human chromosome 20
      Human chromosome 20: entries, gene names and cross-references to MIM
    5. PDB cross-references
      Index of Protein Data Bank (PDB) cross-references
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