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Mouse Anti-AUH Recombinant Antibody (2G12) (CBMAB-A4171-YC)

Provided herein is a Mouse monoclonal antibody against Human AU RNA Binding Methylglutaconyl-CoA Hydratase. The antibody can be used for immunoassay techniques, such as ELISA, WB.
See all AUH antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
2G12
Antibody Isotype
IgG2a, κ
Application
ELISA, WB

Basic Information

Immunogen
Partial recombinant corresponding to aa 44-135 from human AUH (NP_001689) with GST tag.
Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.4
Preservative
None
Concentration
Batch dependent
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
AU RNA binding protein/enoyl-Coenzyme A hydratase
Introduction
AUH is 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 ste
Entrez Gene ID
549
UniProt ID
Q13825
Alternative Names
AU RNA Binding Methylglutaconyl-CoA Hydratase; AU RNA-Binding Protein/Enoyl-Coenzyme A Hydratase; AU RNA Binding Protein/Enoyl-Coenzyme A Hydratase; AU-Specific RNA-Binding Enoyl-CoA Hydratase; AU RNA Binding Protein/Enoyl-CoA Hydratase; AU-Binding Protei
Function
Catalyzes the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA (PubMed:11738050, PubMed:12434311, PubMed:12655555).
Also has itaconyl-CoA hydratase activity by converting itaconyl-CoA into citramalyl-CoA in the C5-dicarboxylate catabolism pathway (PubMed:29056341).
The C5-dicarboxylate catabolism pathway is required to detoxify itaconate, a vitamin B12-poisoning metabolite (PubMed:29056341).
Has very low enoyl-CoA hydratase activity (PubMed:7892223).
Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs (PubMed:7892223).
Biological Process
Branched-chain amino acid catabolic process Source: Reactome
Fatty acid beta-oxidation Source: GO_Central
Leucine catabolic process Source: UniProtKB-UniPathway
Cellular Location
Mitochondrion
Involvement in disease
3-methylglutaconic aciduria 1 (MGCA1): 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. MGCA1 can be distinguished from other forms of MGCA 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 MGCA forms).
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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