HAO1 Human, Active

Hydroxyacid Oxidase 1 Human Recombinant, Active
Cat. No.
BT18773
Source
Escherichia Coli.
Synonyms
Hydroxyacid oxidase 1, HAOX1, Glycolate oxidase, GOX, HAO1, GOX1.
Appearance

Sterile filtered yellowish solution.

Purity
Greater than 95% as determined by SDS-PAGE.
Usage
Prospec's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
Shipped with Ice Packs
In Stock

Description

HAO1 Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 406 amino acids (1-370 a.a) and having a molecular mass of 45.0kDa.

HAO1 is fused to a 36 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Product Specs

Introduction

Glycolate oxidase (HAO1) is an enzyme that belongs to the alpha hydroxy acid oxidases (HAO) family. It plays a crucial role in the oxidation of glycolate to glyoxylate and glyoxylate to oxalate, utilizing FMN as a cofactor and reducing oxygen to hydrogen peroxide. HAO1 is primarily found in the liver and pancreas and exhibits high activity towards two-carbon substrates, particularly glycolate. This enzyme is implicated in hyperoxaluria, a condition characterized by the formation of calcium oxalate kidney stones due to excessive oxalate levels.

Description

Recombinant HAO1, derived from human sources and expressed in E.Coli, is available as a single, non-glycosylated polypeptide chain. This protein consists of 406 amino acids, spanning from position 1 to 370, and has a molecular weight of 45.0kDa. Notably, it includes a 36 amino acid His-tag fused at the N-terminus. The purification process involves proprietary chromatographic techniques to ensure high purity.

Physical Appearance

The product appears as a clear, yellowish solution that has undergone sterile filtration.

Formulation

The HAO1 protein solution is provided at a concentration of 1mg/ml and contains 20% glycerol, 20mM Tris-Hcl (pH8.0), and 0.5M NaCl as components.

Stability

For short-term storage (2-4 weeks), the product can be stored at 4°C. For long-term storage, it is recommended to store the product frozen at -20°C. To further enhance stability during long-term storage, consider adding a carrier protein (0.1% HSA or BSA). It is important to avoid repeated freezing and thawing of the product.

Purity

The purity of the HAO1 protein is determined using SDS-PAGE analysis and is guaranteed to be greater than 95%.

Biological Activity

The specific activity of the enzyme, measured as the amount required to oxidize glyoxylate at pH 8.0 and 25°C, is greater than 3000 pmol/min/ug.

Synonyms
Hydroxyacid oxidase 1, HAOX1, Glycolate oxidase, GOX, HAO1, GOX1.
Source
Escherichia Coli.
Amino Acid Sequence

MRGSHHHHHH GMASMTGGQQ MGRDLYDDDD KDRWGSMLPR LICINDYEQH AKSVLPKSIY DYYRSGANDE ETLADNIAAF SRWKLYPRMLRNVAETDLST SVLGQRVSMP ICVGATAMQR MAHVDGELAT VRACQSLGTG MMLSSWATSS IEEVAEAGPE ALRWLQLYIY KDREVTKKLVRQAEKMGYKA IFVTVDTPYL GNRLDDVRNR FKLPPQLRMK NFETSTLSFS PEENFGDDSG LAAYVAKAID PSISWEDIKW LRRLTSLPIVAKGILRGDDA REAVKHGLNG ILVSNHGARQ LDGVPATIDV LPEIVEAVEG KVEVFLDGGV RKGTDVLKAL ALGAKAVFVG RPIVWGLAFQGEKGVQDVLE ILKEEFRLAM ALSGCQNVKV IDKTLVRKNP LAVSKI.

Product Science Overview

Function and Activity

HAO1 catalyzes the FMN-mediated oxidation of glycolate to glyoxylate and glyoxylate to oxalate, with the reduction of oxygen to hydrogen peroxide . It is most active on two-carbon substrates such as glycolate and is primarily expressed in the liver and pancreas . The enzyme also shows activity on long-chain hydroxy acids like 2-hydroxyhexadecanoate and 2-hydroxyoctanoate, albeit with lower catalytic efficiency .

Genetic and Disease Relevance

HAO1 plays a crucial role in glyoxylate metabolism. Glyoxylate is a highly reactive molecule generated through the oxidation of glycolate in peroxisomes and the catabolism of hydroxyproline in mitochondria . Under normal conditions, glyoxylate is detoxified into glycine by the peroxisomal enzyme alanine-glyoxylate aminotransferase (AGXT) . However, mutations in the AGXT gene can lead to Primary Hyperoxaluria type 1 (PH1), an autosomal recessive disorder characterized by the accumulation of insoluble calcium oxalate stones, primarily in the kidneys .

Recombinant HAO1

Recombinant human HAO1 is produced to study its structure, function, and potential as a therapeutic target. The recombinant form is used in various assays to detect ligand/inhibitor binding and enzyme activity . This research is particularly relevant for developing treatments for metabolic disorders like PH1, where targeting HAO1 can help mitigate the effects of defective AGXT activity .

Therapeutic Potential

Given its role in glyoxylate metabolism, HAO1 is a potential target for therapeutic intervention in conditions like hyperoxaluria, where large deposits of calcium oxalate form kidney stones . By inhibiting HAO1, it may be possible to reduce the accumulation of glyoxylate and, consequently, oxalate, thereby preventing stone formation .

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