Mouse anti-Human AUH Monoclonal Antibody | anti-AUH antibody

AUH (Methylglutaconyl-CoA Hydratase, Mitochondrial, AU-specific RNA-binding Enoyl-CoA Hydratase, AU-binding Protein/Enoyl-CoA Hydratase) APC

Cat. Num. AAA6135454

• Reactivity: Human
• Applications: ELISA, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: AUH; Monoclonal Antibody; AUH (Methylglutaconyl-CoA Hydratase; Mitochondrial; AU-specific RNA-binding Enoyl-CoA Hydratase; AU-binding Protein/Enoyl-CoA Hydratase) APC; anti-AUH antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2a,k
• Clone Number: 2G12
• Specificity: Recognizes human AUH.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Allophycocyanin (APC).

• Applicable Applications for anti-AUH antibody: ELISA (EIA), Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Partial recombinant corresponding to aa44-135 from human AUH (NP_001689) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: RAGPAIWAQGWVPAAGGPAPKRGYSSEMKTEDELRVRHLEEENRGIVVLGINRAYGKNSLSKNLIKMLSKAVDALKSDKKVRTIIIRSEVPG
• Conjugate: APC
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: APC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (35.86kD).)

Testing Data

(Detection limit for recombinant GST tagged AUH is 3ng/ml as a capture antibody.)

Related Product Information for anti-AUH antibody

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.

Product Categories/Family for anti-AUH antibody

Antibodies; Enzymes

NCBI and Uniprot Product Information

• NCBI GI #: 4502327
• NCBI GeneID: 549
• NCBI Accession #: NP_001689
• NCBI GenBank Nucleotide #: NM_001698
• UniProt Accession #: Q13825; Q8WUE4; B1ALV7; B1ALV8
• Molecular Weight: 31.4 kDa (293aa), confirmed by MALDI-TOF
• NCBI Official Full Name: methylglutaconyl-CoA hydratase, mitochondrial isoform 1
• 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