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Protein

Hydroxyacid oxidase 2

Gene

HAO2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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: GO_Central
  • long-chain-(S)-2-hydroxy-long-chain-acid oxidase activity Source: UniProtKB
  • medium-chain-(S)-2-hydroxy-acid oxidase activity Source: UniProtKB-EC
  • signaling receptor binding Source: UniProtKB
  • very-long-chain-(S)-2-hydroxy-acid oxidase activity Source: UniProtKB-EC

GO - Biological processi

Keywordsi

Molecular functionOxidoreductase
LigandFlavoprotein, FMN

Enzyme and pathway databases

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

Organism-specific databases

EuPathDBiHostDB:ENSG00000116882.14
HGNCiHGNC:4810 HAO2
MIMi605176 gene
neXtProtiNX_Q9NYQ3

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

DisGeNETi51179
OpenTargetsiENSG00000116882
PharmGKBiPA29186

Chemistry databases

ChEMBLiCHEMBL2169732
DrugBankiDB03247 Riboflavin Monophosphate

Polymorphism and mutation databases

BioMutaiHAO2
DMDMi13124287

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002063201 – 351Hydroxyacid oxidase 2Add BLAST351

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei178PhosphothreonineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

PaxDbiQ9NYQ3
PeptideAtlasiQ9NYQ3
PRIDEiQ9NYQ3
ProteomicsDBi83262

PTM databases

iPTMnetiQ9NYQ3
PhosphoSitePlusiQ9NYQ3

Expressioni

Tissue specificityi

Liver and kidney.

Gene expression databases

BgeeiENSG00000116882 Expressed in 73 organ(s), highest expression level in right lobe of liver
CleanExiHS_HAO2
ExpressionAtlasiQ9NYQ3 baseline and differential
GenevisibleiQ9NYQ3 HS

Interactioni

Subunit structurei

Homotetramer or homooctamer.By similarity

Binary interactionsi

WithEntry#Exp.IntActNotes
SEC14L4Q9UDX34EBI-8803200,EBI-10320311

GO - Molecular functioni

Protein-protein interaction databases

BioGridi119356, 25 interactors
IntActiQ9NYQ3, 2 interactors
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

Phylogenomic databases

eggNOGiKOG0538 Eukaryota
COG1304 LUCA
GeneTreeiENSGT00390000018717
HOGENOMiHOG000217463
HOVERGENiHBG051881
InParanoidiQ9NYQ3
KOiK11517
OMAiKNEFHTS
OrthoDBiEOG091G0ADA
PhylomeDBiQ9NYQ3
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

Sequences (2+)i

Sequence statusi: Complete.

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

This 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 '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

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
Q5QP02Q5QP02_HUMAN
Hydroxyacid oxidase 2
HAO2
186Annotation score:

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 dbSNP:rs34638261Ensembl.1
Natural variantiVAR_049088221L → M. Corresponds to variant dbSNP:rs6661625Ensembl.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 No translation available.
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

Similar 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
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, 2 interactors
STRINGi9606.ENSP00000316339

Chemistry databases

BindingDBiQ9NYQ3
ChEMBLiCHEMBL2169732
DrugBankiDB03247 Riboflavin Monophosphate

PTM databases

iPTMnetiQ9NYQ3
PhosphoSitePlusiQ9NYQ3

Polymorphism and mutation databases

BioMutaiHAO2
DMDMi13124287

Proteomic databases

PaxDbiQ9NYQ3
PeptideAtlasiQ9NYQ3
PRIDEiQ9NYQ3
ProteomicsDBi83262

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
EuPathDBiHostDB:ENSG00000116882.14
GeneCardsiHAO2
HGNCiHGNC:4810 HAO2
MIMi605176 gene
neXtProtiNX_Q9NYQ3
OpenTargetsiENSG00000116882
PharmGKBiPA29186
GenAtlasiSearch...

Phylogenomic databases

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

Enzyme and pathway databases

ReactomeiR-HSA-390918 Peroxisomal lipid metabolism
R-HSA-9033241 Peroxisomal protein import

Miscellaneous databases

ChiTaRSiHAO2 human
GenomeRNAii51179
PROiPR:Q9NYQ3
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000116882 Expressed in 73 organ(s), highest expression level in right lobe of liver
CleanExiHS_HAO2
ExpressionAtlasiQ9NYQ3 baseline and differential
GenevisibleiQ9NYQ3 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 nameiHAOX2_HUMAN
AccessioniPrimary (citable) accession number: Q9NYQ3
Secondary accession number(s): Q2TU86, Q5QP00, Q9UJS6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 21, 2001
Last sequence update: October 1, 2000
Last modified: November 7, 2018
This is version 147 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

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human chromosome 1
    Human chromosome 1: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
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