Rabbit anti-Human AUH Polyclonal Antibody | anti-AUH antibody

AUH Antibody - N-terminal region

Cat. Num. AAA3219669

• Reactivity: Human
• Applications: Western Blot
• Purity: Affinity Purified
• Synonyms: AUH; Polyclonal Antibody; AUH Antibody - N-terminal region; anti-AUH antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Purity/Purification: Affinity Purified
• Form/Format: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
• Sequence: Synthetic peptide located within the following region: IWAQGWVPAAGGPAPKRGYSSEMKTEDELRVRHLEEENRGIVVLGINRAY
• Sequence Length: 339

• Applicable Applications for anti-AUH antibody: Western Blot (WB)
• Immunogen: The immunogen is a synthetic peptide directed towards the N-terminal region of Human AUH
• Preparation and Storage: For short term use, store at 2-8 degree C up to 1 week. For long term storage, store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Western Blot (WB)

(Host: RabbitTarget Name: AUHSample Type: 293T Whole Cell lysatesAntibody Dilution: 1.0ug/ml)

Related Product Information for anti-AUH antibody

This is a rabbit polyclonal antibody against AUH. It was validated on Western Blot

Target Description: The methylglutaconyl-CoA hydratase, mitochondrial protein binds to the AU-rich element (ARE), a common element found in the 3' UTR of rapidly decaying mRNA such as c-fos, c-myc and granulocyte/ macrophage colony stimulating factor. ARE elements are involved in directing RNA to rapid degradation and deadenylation. AUH is also homologous to enol-CoA hydratase, an enzyme involved in fatty acid degradation, and has been shown to have intrinsic hydratase enzymatic activity. AUH is thus a bifunctional chimera between RNA binding and metabolic enzyme activity. A possible subcellular localization in the mitochondria has been demonstrated for the mouse homolog of this protein which shares 92% identity with the human protein. It has been suggested that AUH may have a novel role as a mitochondrial located AU-binding protein. Human AUH is expressed as a single mRNA species of 1.8 kb, and translated as a 40-kDa precursor protein which is subsequently processed to a 32-kDa mature form.

Product Categories/Family for anti-AUH antibody

Polyclonal; RNA Binding Proteins; Cell Biology; Mitochondria; DNA/RNA/Protein Interactions

NCBI and Uniprot Product Information

• NCBI GI #: 37076898
• NCBI GeneID: 549
• UniProt Accession #: Q13825
• Molecular Weight: 37kDa
• NCBI Official Full Name: Methylglutaconyl-CoA hydratase, mitochondrial
• NCBI Official Synonym Full Names: AU RNA binding methylglutaconyl-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: Mitochondrial; Lyase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 4.2.1.18; RNA-binding

Chromosomal Location of Human Ortholog: 9q22.31

Cellular Component: mitochondrion; mitochondrial matrix

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

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

Disease: 3-methylglutaconic Aciduria, Type I