Names & Taxonomy
- Uniprot ID:
- Q9UJM8
- Entry Name:
- HAOX1_HUMAN
- Status:
- reviewed
- Protein Names:
- Hydroxyacid oxidase 1 (HAOX1) (EC 1.1.3.15) (Glycolate oxidase) (GOX)
- Gene Names:
- HAO1 GOX1 HAOX1
- Gene Names Primary:
- HAO1
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 370
- Sequence:
- MLPRLICINDYEQHAKSVLPKSIYDYYRSGANDEETLADNIAAFSRWKLYPRMLRNVAETDLSTSVLGQRVSMPICVGATAMQRMAHVDGELATVRACQSLGTGMMLSSWATSSIEEVAEAGPEALRWLQLYIYKDREVTKKLVRQAEKMGYKAIFVTVDTPYLGNRLDDVRNRFKLPPQLRMKNFETSTLSFSPEENFGDDSGLAAYVAKAIDPSISWEDIKWLRRLTSLPIVAKGILRGDDAREAVKHGLNGILVSNHGARQLDGVPATIDVLPEIVEAVEGKVEVFLDGGVRKGTDVLKALALGAKAVFVGRPIVWGLAFQGEKGVQDVLEILKEEFRLAMALSGCQNVKVIDKTLVRKNPLAVSKI
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Peroxisome.
Function
- 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.
- Pathway:
- Organic acid metabolism; glycolate degradation; 3-phospho-D-glycerate from glycolate: step 1/4.
- Catalytic Activity:
- (S)-2-hydroxy acid + O(2) = 2-oxo acid + H(2)O(2).
- Cofactor:
- COFACTOR: Name=FMN; Xref=ChEBI:CHEBI:58210;
- Kinetics:
- BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=141 uM for glycolate
- Active Site:
- ACT_SITE 260 260 Proton acceptor.
- Gene Ontology Go:
- peroxisomal matrix
peroxisome
(S)-2-hydroxy-acid oxidase activity
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
receptor binding
very-long-chain-(S)-2-hydroxy-acid oxidase activity
cellular nitrogen compound metabolic process
fatty acid alpha-oxidation
glycolate catabolic process
glyoxylate metabolic process
response to oxidative stress
small molecule metabolic process - Gene Ontology Biological Process:
- cellular nitrogen compound metabolic process
fatty acid alpha-oxidation
glycolate catabolic process
glyoxylate metabolic process
response to oxidative stress
small molecule metabolic process - Gene Ontology Molecular Function:
- (S)-2-hydroxy-acid oxidase activity
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
receptor binding
very-long-chain-(S)-2-hydroxy-acid oxidase activity - Gene Ontology Cellular Component:
- peroxisomal matrix
peroxisome - Keywords:
- 3D-structure
Complete proteome
FMN
Flavoprotein
Oxidoreductase
Peroxisome
Phosphoprotein
Reference proteome