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Rabbit Anti-HAO1/FITC Conjugated antibody
background:
GOX is a 370 amino acid protein that is expressed in liver and pancreas. HAO1 is localized to peroxisomes and aids in organic acid metabolism via 2-hydroxyacid oxidase activity. 2-hydroxyacid oxidases, such as HAO1, are enzymes that require a flavin cofactor to oxidize 2-hydroxyacids to 2-ketoacids while reducing oxygen to hydrogen peroxide. HAO1 prefenentially oxidizes the substrate glycolate and also oxidizes other substrates, including 2-hydroxy fatty acids as well as L-?hydroxy acids of moderately short chain lengths. The oxidation of glycolate yields glyoxylate which is utilized for peroxisomal synthesis of glycine. HAO1 is also able to convert glyoxylate to oxalate. HAO1 is thought to play a role in the pathophysiology of hyperoxaluria type 1, which is caused by defects in AGXT, a peroxisomal enzyme, leading to accumulation of glyoxylate. Hyperoxaluria type 1 is characterized by an accumulation of oxalate that is thought to lead to precipitates of calcium oxalate in kidneys which can be fatal.
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.
Subcellular Location:
Peroxisome.
Tissue Specificity:
Liver.
Similarity:
Belongs to the FMN-dependent alpha-hydroxy acid dehydrogenase family.
Contains 1 FMN hydroxy acid dehydrogenase domain.
Database links:
Entrez Gene: 54363 Human
Entrez Gene: 15112 Mouse
Omim: 605023 Human
SwissProt: Q9UJM8 Human
SwissProt: Q9WU19 Mouse
Unigene: 193128 Human
Unigene: 26634 Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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