HAO1

HAO1 has 2-hydroxyacid oxidase activity. Most active on the 2-carbon substrate glycolate, but HAO1 is also active on 2-hydroxy fatty acids, with high activity towards 2-hydroxy palmitate and 2-hydroxy octanoate.

HAO1 Related Areas

Enzyme>>Oxidoreductase>>HAO1

HAO1 Alternative Names

HAO1, GOX, GOX1, HAOX1, MGC142225, MGC142227 [Homo sapiens]

Hao1, HAOX1, RP23-376O20.1, GOX, Gox1, Hao-1, MGC141211 [Mus musculus]

Summaries for HAO1

Entrez Gene summary for HAO1:

This HAO1 gene is one of three related genes that have 2-hydroxyacid oxidase activity yet differ in encoded protein amino acid sequence, tissue expression and substrate preference. Subcellular location of HAO1 is the peroxisome. Specifically, this gene is expressed primarily in liver and pancreas and the encoded protein is most active on glycolate, a two-carbon substrate. HAO1 is also active on 2-hydroxy fatty acids. The transcript detected at high levels in pancreas may represent an alternatively spliced form or the use of a multiple near-consensus upstream polyadenylation site. [provided by RefSeq, Jul 2008]

OMIM - description for HAO1:

2-Hydroxyacid oxidases can oxidize a broad range of 2-hydroxyacids, ranging from glycolate to long-chain 2-hydroxy fatty acids such as 2-hydroxypalmitate. These enzymes utilize a flavin cofactor and convert 2-hydroxyacids to 2-ketoacids with the concomitant reduction of molecular oxygen to hydrogen peroxide.

Human HAO1 Protein General Information

 

• Protein names: Hydroxyacid oxidase 1, Short name=HAOX1
• Sequence length: 370 AA.
• Catalytic activity: (S)-2-hydroxy acid + O2 = 2-oxo acid + H2O2.
• Sequence similarities: HAO1 belongs to the FMN-dependent alpha-hydroxy acid dehydrogenase family. HAO1 contains 1 FMN hydroxy acid dehydrogenase domain.
• Cofactor: FMN.
• Pathway: Organic acid metabolism; glycolate degradation; 3-phospho-D-glycerate from glycolate: step 1/4.
• Subcellular location: Peroxisome.
• Tissue specificity: Liver.
• Biophysicochemical properties: Kinetic parameters:
  KM=141 µM for glycolate
  KM=40 µM for 2-hydroxy octanoate
  KM=2200 µM for glyoxylate

General information above from UniProt

Function for HAO1 Protein

UniProtKB:

HAO1 has 2-hydroxyacid oxidase activity. Most active on the 2-carbon substrate glycolate, but HAO1 is also active on 2-hydroxy fatty acids, with high activity towards 2-hydroxy palmitate and 2-hydroxy octanoate.

Genatlas:

• hydroxyacid oxidase 1, glycolate oxidase