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

Name: Mouse Anti-AUH Recombinant Antibody (2G12)
SKU: CBMAB-A4171-YC
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Mouse

Clone

2G12

Application

ELISA, WB

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.

More Infomation

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Isotype control

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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