AUH Human

AU RNA Binding Protein/Enoyl-CoA Hydratase Human Recombinant
Cat. No.
BT25537
Source
Escherichia Coli.
Synonyms
Methylglutaconyl-CoA hydratase, mitochondrial, AU-specific RNA-binding enoyl-CoA hydratase, AU-binding protein/enoyl-CoA hydratase, AUH.
Appearance
Sterile Filtered colorless solution.
Purity
Greater than 95.0% as determined by SDS-PAGE.
Usage
THE BioTek'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.
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Description

AUH Human Recombinant fused with a 21 amino acid His tag at N-terminus produced in E.Coli is a single, non-glycosylated, polypeptide chain containing 293 amino acids (68-339 a.a.) and having a molecular mass of 31.4kDa. The AUH is purified by proprietary chromatographic techniques.

Product Specs

Introduction
Mitochondrial methylglutaconyl-CoA hydratase (AUH) is an enzyme that plays a role in the breakdown of amino acids. It facilitates the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA and water. In humans, AUH is transcribed from a single 1.8 kb mRNA molecule and translated into a 40kDa precursor protein. This precursor protein is then processed into a mature 32kDa form. AUH exhibits minimal enoyl-CoA hydratase activity. The AUH protein interacts with the AU-rich element (ARE), a sequence commonly found in the 3' untranslated region (UTR) of mRNAs with rapid decay rates, such as those encoding c-fos, c-myc, and granulocyte/macrophage colony-stimulating factor. AU-rich elements are involved in targeting RNA for rapid degradation and deadenylation. Additionally, AUH shares homology with enol-CoA hydratase, an enzyme implicated in fatty acid degradation, and has demonstrated inherent hydratase enzymatic activity. Consequently, AUH acts as a bifunctional molecule, bridging RNA binding and metabolic enzyme activity.
Description
Recombinant AUH Human, fused with a 21 amino acid His tag at its N-terminus, is produced in E. coli. It is a single, non-glycosylated polypeptide chain composed of 293 amino acids (residues 68-339) and possesses a molecular weight of 31.4kDa. The purification of AUH is achieved through proprietary chromatographic methods.
Physical Appearance
A clear, colorless solution that has been sterilized by filtration.
Formulation
The AUH solution is supplied at a concentration of 1 mg/ml and contains 20mM Tris-HCl buffer (pH 8.0), 20% glycerol, 0.1M NaCl, and 1mM DTT.
Stability
For short-term storage (up to 4 weeks), the AUH solution should be kept at 4°C. For longer-term storage, it is recommended to freeze the solution at -20°C. To further enhance stability during long-term storage, consider adding a carrier protein like HSA or BSA (0.1%). Repeated freezing and thawing of the solution should be avoided.
Purity
The purity of AUH is greater than 95%, as determined by SDS-PAGE analysis.
Synonyms
Methylglutaconyl-CoA hydratase, mitochondrial, AU-specific RNA-binding enoyl-CoA hydratase, AU-binding protein/enoyl-CoA hydratase, AUH.
Source
Escherichia Coli.
Amino Acid Sequence

MGSSHHHHHH SSGLVPRGSH MSSEMKTEDE LRVRHLEEEN RGIVVLGINR AYGKNSLSKN LIKMLSKAVD ALKSDKKVRT IIIRSEVPGI FCAGADLKER AKMSSSEVGP FVSKIRAVIN DIANLPVPTI AAIDGLALGG GLELALACDI RVAASSAKMG LVETKLAIIP GGGGTQRLPR AIGMSLAKEL IFSARVLDGK EAKAVGLISH VLEQNQEGDA AYRKALDLAR EFLPQGPVAM RVAKLAINQG MEVDLVTGLA IEEACYAQTI PTKDRLEGLL AFKEKRPPRY KGE.

Product Science Overview

Structure and Function

AUH is a member of the enoyl-CoA hydratase/isomerase superfamily, but it is unique in its ability to bind RNA . The protein has two distinct functional domains:

  1. RNA-Binding Domain: This domain allows AUH to bind to AU-rich elements (AREs) found in the 3’ untranslated regions (UTRs) of rapidly decaying mRNAs, such as c-fos, c-myc, and granulocyte/macrophage colony-stimulating factor . AREs are involved in directing RNA to rapid degradation and deadenylation.
  2. Hydratase Domain: This domain exhibits enoyl-CoA hydratase activity, specifically catalyzing the hydration of 3-methylglutaconyl-CoA to 3-hydroxy-3-methyl-glutaryl-CoA, a critical step in the leucine degradation pathway .
Biological Significance

AUH is localized to the mitochondrial matrix and the inner mitochondrial membrane, where it may be involved in mitochondrial protein synthesis . The dual functionality of AUH makes it a key player in both RNA metabolism and mitochondrial function.

Clinical Relevance

Mutations in the AUH gene are associated with a rare metabolic disorder known as 3-methylglutaconic aciduria, type I . This condition is characterized by elevated levels of 3-methylglutaconic acid in the urine, leading to various clinical symptoms, including developmental delay, muscle weakness, and neurological abnormalities.

Research and Applications

The recombinant form of AUH has been extensively studied to understand its dual functionality and potential therapeutic applications. Research has shown that the recombinant protein can bind specifically to AU-rich transcripts and exhibit enzymatic activity, making it a valuable tool for studying RNA metabolism and mitochondrial function .

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