AUH Antibody

Datasheet

Code	CSB-PA621698ESR2HU
Size	US$166
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Image	Western blot All lanes: Methylglutaconyl-CoA hydratase, mitochondrial antibody at 6μg/ml Lane 1: Rat brain tissue Lane 2: Rat kidney tissue Secondary Goat polyclonal to rabbit IgG at 1/10000 dilution Predicted band size: 36, 33 kDa Observed band size: 36 kDa Immunohistochemistry of paraffin-embedded human tonsil tissue using CSB-PA621698ESR2HU at dilution of 1: 100 Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA621698ESR2HU at dilution of 1: 100
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Product Details
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Target Background

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) AUH Polyclonal antibody

Uniprot No.

Q13825

Target Names

AUH

Alternative Names

AUHMethylglutaconyl-CoA hydratase antibody; mitochondrial antibody; EC 4.2.1.18 antibody; AU-specific RNA-binding enoyl-CoA hydratase antibody; AU-binding protein/enoyl-CoA hydratase antibody; Itaconyl-CoA hydratase antibody; EC 4.2.1.56 antibody

Raised in

Rabbit

Species Reactivity

Human, Rat

Immunogen

Recombinant Human Methylglutaconyl-CoA hydratase, mitochondrial protein (68-339AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Clonality

Polyclonal

Isotype

IgG

Purification Method

Antigen Affinity Purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Form

Liquid

Tested Applications

ELISA, WB, IHC

Recommended Dilution

Application	Recommended Dilution
WB	1:500-1:2000
IHC	1:20-1:200

Protocols

ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Customer Reviews and Q&A

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Target Background

Function

Catalyzes the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA. Also has itaconyl-CoA hydratase activity by converting itaconyl-CoA into citramalyl-CoA in the C5-dicarboxylate catabolism pathway. The C5-dicarboxylate catabolism pathway is required to detoxify itaconate, a vitamin B12-poisoning metabolite. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs.

Gene References into Functions

AUH localizes to the inner mitochondrial membrane and matrix where it associates with mitochondrial ribosomes and regulates protein synthesis. PMID: 24598254
Phenotypic heterogeneity in two siblings with 3-methylglutaconic aciduria type I caused by a novel deletion of exons 1-3 within the AUH gene. PMID: 21840233
3-Methylglutaconic aciduria type I is caused by mutations in AUH PMID: 12434311
Human 3-methylglutaconyl-CoA hydratase is identical with RNA-binding protein (AUH); molecular analyses of MGA1 patients show homozygosity or compound heterozygosity for mutations in AUH. PMID: 12655555
Mutation analysis in the AUH gene revealed homozygosity for a novel splice site mutation IVS9-2A>G. We conclude that MGA1 may be associated with fever-associated seizures even in children without delayed psychomotor development. PMID: 15033206
Mutations in the AUH gene are linked to metabolic disease 3-methylglutaconic aciduria type I (MGA1). PMID: 16640564
The AUH trimer dimerizes upon binding to one molecule of a long RNA containing 24 repeats of the AUUU motif, (AUUU)(24)A. PMID: 18831052

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Involvement in disease

3-methylglutaconic aciduria 1 (MGA1)

Subcellular Location

Mitochondrion.

Protein Families

Enoyl-CoA hydratase/isomerase family

Database Links

HGNC: 890

OMIM: 250950

KEGG: hsa:549

STRING: 9606.ENSP00000364883

UniGene: Hs.175905

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Antibody FAQs What Is an Antibody? How to Choose an Antibody for Scientific Research? The difference between primary antibody and secondary antibod The Meaning of Irregular Antibody Screening Tag Antibodies, Your Good Partner in Protein Research How to Choose the Loading Control Antibodies?

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