Hmdb loader
Identification
HMDB Protein ID HMDBP00787
Secondary Accession Numbers
  • 6067
  • HMDBP03571
Name Hydroxyacid oxidase 1
Synonyms
  1. GOX
  2. Glycolate oxidase
  3. HAOX1
Gene Name HAO1
Protein Type Unknown
Biological Properties
General Function Involved in FMN binding
Specific Function 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.
Pathways
  • glycolate degradation
  • Glyoxylate and dicarboxylate metabolism
  • Peroxisome
Reactions
(S)-2-hydroxy acid + Oxygen → 2-oxo acid + Hydrogen peroxide details
Glycolic acid + Oxygen → Glyoxylic acid + Hydrogen peroxide details
GO Classification
Biological Process
cellular nitrogen compound metabolic process
glyoxylate metabolic process
response to oxidative stress
fatty acid alpha-oxidation
glycolate catabolic process
Cellular Component
peroxisomal matrix
Function
binding
nucleotide binding
catalytic activity
fmn binding
oxidoreductase activity
Molecular Function
FMN binding
glycolate oxidase activity
glyoxylate oxidase activity
long-chain-(S)-2-hydroxy-long-chain-acid oxidase activity
medium-chain-(S)-2-hydroxy-acid oxidase activity
very-long-chain-(S)-2-hydroxy-acid oxidase activity
Process
metabolic process
oxidation reduction
Cellular Location
  1. Peroxisome
Gene Properties
Chromosome Location 20
Locus 20p12
SNPs HAO1
Gene Sequence
>1113 bp
ATGCTCCCCCGGCTAATTTGTATCAATGATTATGAACAACATGCTAAATCAGTACTTCCA
AAGTCTATATATGACTATTACAGGTCTGGGGCAAATGATGAAGAAACTTTGGCTGATAAT
ATTGCAGCATTTTCCAGATGGAAGCTGTATCCAAGGATGCTCCGGAATGTTGCTGAAACA
GATCTGTCGACTTCTGTTTTAGGACAGAGGGTCAGCATGCCAATATGTGTGGGGGCTACG
GCCATGCAGCGCATGGCTCATGTGGACGGCGAGCTTGCCACTGTGAGAGCCTGTCAGTCC
CTGGGAACGGGCATGATGTTGAGTTCCTGGGCCACCTCCTCAATTGAAGAAGTGGCGGAA
GCTGGTCCTGAGGCACTTCGTTGGCTGCAACTGTATATCTACAAGGACCGAGAAGTCACC
AAGAAGCTAGTGCGGCAGGCAGAGAAGATGGGCTACAAGGCCATATTTGTGACAGTGGAC
ACACCTTACCTGGGCAACCGTCTGGATGATGTGCGTAACAGATTCAAACTGCCGCCACAA
CTCAGGATGAAAAATTTTGAAACCAGTACTTTATCATTTTCTCCTGAGGAAAATTTTGGA
GACGACAGTGGACTTGCTGCATATGTGGCTAAAGCAATAGACCCATCTATCAGCTGGGAA
GATATCAAATGGCTGAGAAGACTGACATCATTGCCAATTGTTGCAAAGGGCATTTTGAGA
GGTGATGATGCCAGGGAGGCTGTTAAACATGGCTTGAATGGGATCTTGGTGTCGAATCAT
GGGGCTCGACAACTCGATGGGGTGCCAGCCACTATTGATGTTCTGCCAGAAATTGTGGAG
GCTGTGGAAGGGAAGGTGGAAGTCTTCCTGGACGGGGGTGTGCGGAAAGGCACTGATGTT
CTGAAAGCTCTGGCTCTTGGCGCCAAGGCTGTGTTTGTGGGGAGACCAATCGTTTGGGGC
TTAGCTTTCCAGGGGGAGAAAGGTGTTCAAGATGTCCTCGAGATACTAAAGGAAGAATTC
CGGTTGGCCATGGCTCTGAGTGGGTGCCAGAATGTGAAAGTCATCGACAAGACATTGGTG
AGGAAAAATCCTTTGGCCGTTTCCAAGATCTGA
Protein Properties
Number of Residues 370
Molecular Weight 40923.945
Theoretical pI 8.096
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Hydroxyacid oxidase 1
MLPRLICINDYEQHAKSVLPKSIYDYYRSGANDEETLADNIAAFSRWKLYPRMLRNVAET
DLSTSVLGQRVSMPICVGATAMQRMAHVDGELATVRACQSLGTGMMLSSWATSSIEEVAE
AGPEALRWLQLYIYKDREVTKKLVRQAEKMGYKAIFVTVDTPYLGNRLDDVRNRFKLPPQ
LRMKNFETSTLSFSPEENFGDDSGLAAYVAKAIDPSISWEDIKWLRRLTSLPIVAKGILR
GDDAREAVKHGLNGILVSNHGARQLDGVPATIDVLPEIVEAVEGKVEVFLDGGVRKGTDV
LKALALGAKAVFVGRPIVWGLAFQGEKGVQDVLEILKEEFRLAMALSGCQNVKVIDKTLV
RKNPLAVSKI
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID Q9UJM8
UniProtKB/Swiss-Prot Entry Name HAOX1_HUMAN
PDB IDs
GenBank Gene ID AF244134
GeneCard ID HAO1
GenAtlas ID HAO1
HGNC ID HGNC:4809
References
General References
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  3. Williams E, Cregeen D, Rumsby G: Identification and expression of a cDNA for human glycolate oxidase. Biochim Biophys Acta. 2000 Sep 7;1493(1-2):246-8. [PubMed:10978532 ]
  4. Jones JM, Morrell JC, Gould SJ: Identification and characterization of HAOX1, HAOX2, and HAOX3, three human peroxisomal 2-hydroxy acid oxidases. J Biol Chem. 2000 Apr 28;275(17):12590-7. [PubMed:10777549 ]
  5. Murray MS, Holmes RP, Lowther WT: Active site and loop 4 movements within human glycolate oxidase: implications for substrate specificity and drug design. Biochemistry. 2008 Feb 26;47(8):2439-49. doi: 10.1021/bi701710r. Epub 2008 Jan 24. [PubMed:18215067 ]
  6. Bourhis JM, Vignaud C, Pietrancosta N, Gueritte F, Guenard D, Lederer F, Lindqvist Y: Structure of human glycolate oxidase in complex with the inhibitor 4-carboxy-5-[(4-chlorophenyl)sulfanyl]-1,2,3-thiadiazole. Acta Crystallogr Sect F Struct Biol Cryst Commun. 2009 Dec 1;65(Pt 12):1246-53. doi: 10.1107/S1744309109041670. Epub 2009 Nov 27. [PubMed:20054120 ]