Rabbit AUH Polyclonal Antibody | anti-AUH antibody

Anti-AUH Antibody

Cat. Num. AAA8245303

• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry
• Purity: The antibody was purified by immunogen affinity chromatography.
• Synonyms: AUH; Polyclonal Antibody; Anti-AUH Antibody; Methylglutaconyl-CoA hydratase; mitochondrial; AU-specific RNA-binding enoyl-CoA hydratase; AU-binding protein/enoyl-CoA hydratase; anti-AUH antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Specificity: Recognizes endogenous levels of AUH protein.
• Purity/Purification: The antibody was purified by immunogen affinity chromatography.
• Form/Format: Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.
• Sequence Length: 310

• Applicable Applications for anti-AUH antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB: 1/500 - 1/2000; IHC: 1/50 - 1/200
• Immunogen: Recombinant full length protein of human AUH
• Preparation and Storage: Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.

Western Blot (WB)

(Western blot analysis of AUH expression in A549 (A), SW480 (B), mouse brain (C), mouse kidney (D), rat heart (E) whole cell lysates.)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of AUH staining in rat kidney formalin fixed paraffin embedded tissue section. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH 6.0). The section was then incubated with the antibody at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.)

Related Product Information for anti-AUH antibody

Rabbit polyclonal antibody to AUH

NCBI and Uniprot Product Information

• NCBI GI #: 807201170
• NCBI GeneID: 549
• NCBI Accession #: NP_001293119.1
• NCBI GenBank Nucleotide #: NM_001306190.1
• UniProt Accession #: Q13825; Q8WUE4; B1ALV7; B1ALV8
• Molecular Weight: 32,508 Da
• NCBI Official Full Name: methylglutaconyl-CoA hydratase, mitochondrial isoform 2
• NCBI Official Synonym Full Names: AU RNA binding protein/enoyl-CoA hydratase
• NCBI Official Symbol: AUH
• NCBI Protein Information: methylglutaconyl-CoA hydratase, mitochondrial
• UniProt Protein Name: Methylglutaconyl-CoA hydratase, mitochondrial
• Protein Family: Methylglutaconyl-CoA hydratase
• UniProt Gene Name: AUH
• UniProt Synonym Gene Names: AU-binding protein/enoyl-CoA hydratase
• UniProt Entry Name: AUHM_HUMAN

NCBI Description

This gene encodes bifunctional mitochondrial protein that has both RNA-binding and hydratase activities. The encoded protein is a methylglutaconyl-CoA hydratase that catalyzes the hydration of 3-methylglutaconyl-CoA to 3-hydroxy-3-methyl-glutaryl-CoA, a critical step in the leucine degradation pathway. This protein also binds AU-rich elements (AREs) found in the 3' UTRs of rapidly decaying mRNAs including c-fos, c-myc and granulocyte/ macrophage colony stimulating factor. ARE elements are involved in directing RNA to rapid degradation and deadenylation. This protein is localizes to the mitochondrial matrix and the inner mitochondrial membrane and may be involved in mitochondrial protein synthesis. Mutations in this gene are the cause of 3-methylglutaconic aciduria, type I. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2015]

Uniprot Description

AUH: Catalyzes the conversion of 3-methylglutaconyl-CoA to 3- hydroxy-3-methylglutaryl-CoA. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs. Defects in AUH are the cause of 3-methylglutaconic aciduria type 1 (MGA1). MGA1 is an inborn error of leucine metabolism. It leads to an autosomal recessive syndrome with variable clinical phenotype, ranging from delayed speech development to severe psychomotor retardation, coma, failure to thrive, metabolic acidosis and dystonia. MGA1 can be distinguished from other forms of MGA by the pattern of metabolite excretion: 3- methylglutaconic acid levels are higher than those detected in other forms, whereas methylglutaric acid levels are usually only slightly elevated, and there is a high level of 3- hydroxyisovaleric acid excretion (not present in other MGA forms). Belongs to the enoyl-CoA hydratase/isomerase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: RNA-binding; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Lyase; EC 4.2.1.18; Mitochondrial

Chromosomal Location of Human Ortholog: 9q22.31

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function: enoyl-CoA hydratase activity; methylglutaconyl-CoA hydratase activity; mRNA 3'-UTR binding

Biological Process: branched chain family amino acid catabolic process; leucine catabolic process

Disease: 3-methylglutaconic Aciduria, Type I