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Protein

Hydroxyacid oxidase 2

Gene

HAO2

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

Functioni

Catalyzes the oxidation of L-alpha-hydroxy acids as well as, more slowly, that of L-alpha-amino acids.

Catalytic activityi

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

Cofactori

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei106FMNPROSITE-ProRule annotation1
Binding sitei128FMNPROSITE-ProRule annotation1
Binding sitei130SubstratePROSITE-ProRule annotation1
Binding sitei156FMNPROSITE-ProRule annotation1
Binding sitei165SubstratePROSITE-ProRule annotation1
Binding sitei222FMNPROSITE-ProRule annotation1
Active sitei246Proton acceptorPROSITE-ProRule annotation1
Binding sitei249SubstratePROSITE-ProRule annotation1
Binding sitei301FMNPROSITE-ProRule annotation1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi277 – 301FMNPROSITE-ProRule annotationAdd BLAST25

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
  • receptor binding Source: UniProtKB
  • very-long-chain-(S)-2-hydroxy-acid oxidase activity Source: UniProtKB-EC

GO - Biological processi

  • cellular lipid metabolic process Source: Reactome
  • fatty acid oxidation Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Ligandi

Flavoprotein, FMN

Enzyme and pathway databases

BioCyciZFISH:HS04066-MONOMER.
ReactomeiR-HSA-390918. Peroxisomal lipid metabolism.

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
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

HGNCiHGNC:4810. HAO2.

Subcellular locationi

GO - Cellular componenti

  • extracellular exosome Source: UniProtKB
  • mitochondrion Source: Ensembl
  • peroxisomal matrix Source: Reactome
  • peroxisome Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Peroxisome

Pathology & Biotechi

Organism-specific databases

DisGeNETi51179.
OpenTargetsiENSG00000116882.
PharmGKBiPA29186.

Chemistry databases

ChEMBLiCHEMBL2169732.

Polymorphism and mutation databases

BioMutaiHAO2.
DMDMi13124287.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedBy similarity
ChainiPRO_00002063202 – 351Hydroxyacid oxidase 2Add BLAST350

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei178PhosphothreonineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiQ9NYQ3.
PaxDbiQ9NYQ3.
PeptideAtlasiQ9NYQ3.
PRIDEiQ9NYQ3.

PTM databases

iPTMnetiQ9NYQ3.
PhosphoSitePlusiQ9NYQ3.

Expressioni

Tissue specificityi

Liver and kidney.

Gene expression databases

BgeeiENSG00000116882.
CleanExiHS_HAO2.
ExpressionAtlasiQ9NYQ3. baseline and differential.
GenevisibleiQ9NYQ3. HS.

Interactioni

Subunit structurei

Homotetramer or homooctamer.By similarity

GO - Molecular functioni

  • receptor binding Source: UniProtKB

Protein-protein interaction databases

BioGridi119356. 25 interactors.
IntActiQ9NYQ3. 1 interactor.
STRINGi9606.ENSP00000316339.

Chemistry databases

BindingDBiQ9NYQ3.

Structurei

3D structure databases

ProteinModelPortaliQ9NYQ3.
SMRiQ9NYQ3.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini2 – 351FMN hydroxy acid dehydrogenasePROSITE-ProRule annotationAdd BLAST350

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi349 – 351Microbody targeting signalSequence analysis3

Sequence similaritiesi

Belongs to the FMN-dependent alpha-hydroxy acid dehydrogenase family.PROSITE-ProRule annotation
Contains 1 FMN hydroxy acid dehydrogenase domain.PROSITE-ProRule annotation

Phylogenomic databases

eggNOGiKOG0538. Eukaryota.
COG1304. LUCA.
GeneTreeiENSGT00390000018717.
HOGENOMiHOG000217463.
HOVERGENiHBG051881.
InParanoidiQ9NYQ3.
KOiK11517.
OMAiWNDLSWF.
OrthoDBiEOG091G0ADA.
PhylomeDBiQ9NYQ3.
TreeFamiTF313363.

Family and domain databases

Gene3Di3.20.20.70. 1 hit.
InterProiIPR013785. Aldolase_TIM.
IPR012133. Alpha-hydoxy_acid_DH_FMN.
IPR000262. FMN-dep_DH.
IPR008259. FMN_hydac_DH_AS.
[Graphical view]
PfamiPF01070. FMN_dh. 1 hit.
[Graphical view]
PIRSFiPIRSF000138. Al-hdrx_acd_dh. 1 hit.
PROSITEiPS00557. FMN_HYDROXY_ACID_DH_1. 1 hit.
PS51349. FMN_HYDROXY_ACID_DH_2. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

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

Isoform 1 (identifier: Q9NYQ3-1) [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
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
Length:351
Mass (Da):38,839
Last modified:October 1, 2000 - v1
Checksum:i7330DE44E282947D
GO
Isoform 2 (identifier: Q9NYQ3-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-1: M → MEDKMWSECEGPEM

Note: No experimental confirmation available.
Show »
Length:364
Mass (Da):40,391
Checksum:i13F52C5D431E8B2E
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti80F → Y in AAF14000 (Ref. 2) Curated1
Sequence conflicti292 – 294LGA → HED in AAF14000 (Ref. 2) Curated3

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_04908715E → K.Corresponds to variant rs34638261dbSNPEnsembl.1
Natural variantiVAR_049088221L → M.Corresponds to variant rs6661625dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0550951M → MEDKMWSECEGPEM in isoform 2. 1 Publication1

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. Translation: CAC19798.1.
CH471122 Genomic DNA. Translation: EAW56698.1.
CH471122 Genomic DNA. Translation: EAW56699.1.
BC020863 mRNA. Translation: AAH20863.1.
CCDSiCCDS76196.1. [Q9NYQ3-2]
CCDS901.1. [Q9NYQ3-1]
RefSeqiNP_001005783.2. NM_001005783.2. [Q9NYQ3-2]
NP_057611.1. NM_016527.3. [Q9NYQ3-1]
UniGeneiHs.659767.

Genome annotation databases

EnsembliENST00000325945; ENSP00000316339; ENSG00000116882. [Q9NYQ3-1]
ENST00000361035; ENSP00000354314; ENSG00000116882. [Q9NYQ3-2]
ENST00000622548; ENSP00000483507; ENSG00000116882. [Q9NYQ3-1]
GeneIDi51179.
KEGGihsa:51179.
UCSCiuc001ehq.1. human. [Q9NYQ3-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

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. Translation: CAC19798.1.
CH471122 Genomic DNA. Translation: EAW56698.1.
CH471122 Genomic DNA. Translation: EAW56699.1.
BC020863 mRNA. Translation: AAH20863.1.
CCDSiCCDS76196.1. [Q9NYQ3-2]
CCDS901.1. [Q9NYQ3-1]
RefSeqiNP_001005783.2. NM_001005783.2. [Q9NYQ3-2]
NP_057611.1. NM_016527.3. [Q9NYQ3-1]
UniGeneiHs.659767.

3D structure databases

ProteinModelPortaliQ9NYQ3.
SMRiQ9NYQ3.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi119356. 25 interactors.
IntActiQ9NYQ3. 1 interactor.
STRINGi9606.ENSP00000316339.

Chemistry databases

BindingDBiQ9NYQ3.
ChEMBLiCHEMBL2169732.

PTM databases

iPTMnetiQ9NYQ3.
PhosphoSitePlusiQ9NYQ3.

Polymorphism and mutation databases

BioMutaiHAO2.
DMDMi13124287.

Proteomic databases

EPDiQ9NYQ3.
PaxDbiQ9NYQ3.
PeptideAtlasiQ9NYQ3.
PRIDEiQ9NYQ3.

Protocols and materials databases

DNASUi51179.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000325945; ENSP00000316339; ENSG00000116882. [Q9NYQ3-1]
ENST00000361035; ENSP00000354314; ENSG00000116882. [Q9NYQ3-2]
ENST00000622548; ENSP00000483507; ENSG00000116882. [Q9NYQ3-1]
GeneIDi51179.
KEGGihsa:51179.
UCSCiuc001ehq.1. human. [Q9NYQ3-1]

Organism-specific databases

CTDi51179.
DisGeNETi51179.
GeneCardsiHAO2.
HGNCiHGNC:4810. HAO2.
MIMi605176. gene.
neXtProtiNX_Q9NYQ3.
OpenTargetsiENSG00000116882.
PharmGKBiPA29186.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0538. Eukaryota.
COG1304. LUCA.
GeneTreeiENSGT00390000018717.
HOGENOMiHOG000217463.
HOVERGENiHBG051881.
InParanoidiQ9NYQ3.
KOiK11517.
OMAiWNDLSWF.
OrthoDBiEOG091G0ADA.
PhylomeDBiQ9NYQ3.
TreeFamiTF313363.

Enzyme and pathway databases

BioCyciZFISH:HS04066-MONOMER.
ReactomeiR-HSA-390918. Peroxisomal lipid metabolism.

Miscellaneous databases

ChiTaRSiHAO2. human.
GenomeRNAii51179.
PROiQ9NYQ3.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000116882.
CleanExiHS_HAO2.
ExpressionAtlasiQ9NYQ3. baseline and differential.
GenevisibleiQ9NYQ3. HS.

Family and domain databases

Gene3Di3.20.20.70. 1 hit.
InterProiIPR013785. Aldolase_TIM.
IPR012133. Alpha-hydoxy_acid_DH_FMN.
IPR000262. FMN-dep_DH.
IPR008259. FMN_hydac_DH_AS.
[Graphical view]
PfamiPF01070. FMN_dh. 1 hit.
[Graphical view]
PIRSFiPIRSF000138. Al-hdrx_acd_dh. 1 hit.
PROSITEiPS00557. FMN_HYDROXY_ACID_DH_1. 1 hit.
PS51349. FMN_HYDROXY_ACID_DH_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiHAOX2_HUMAN
AccessioniPrimary (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: November 2, 2016
This is version 133 of the entry and version 1 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

Complete proteome, Reference proteome

Documents

  1. Human chromosome 1
    Human chromosome 1: 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. 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.