AUH Antibody

Code CSB-PA002451GA01HU
Size $600
Order now
Have Questions? Leave a Message or Start an on-line Chat

Product Details

Uniprot No.
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,Mouse,Rat
Immunogen
Human AUH
Immunogen Species
Homo sapiens (Human)
Isotype
IgG
Purification Method
Antigen Affinity purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications
ELISA,WB,IHC
Troubleshooting and 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

 Customer Reviews

There are currently no reviews for this product.

Submit a Review here

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
  1. AUH localizes to the inner mitochondrial membrane and matrix where it associates with mitochondrial ribosomes and regulates protein synthesis. PMID: 24598254
  2. 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. 3-Methylglutaconic aciduria type I is caused by mutations in AUH PMID: 12434311
  4. 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
  5. 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
  6. Mutations in the AUH gene are linked to metabolic disease 3-methylglutaconic aciduria type I (MGA1). PMID: 16640564
  7. 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

Show More

Hide All

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

icon of phone
Call us
301-363-4651 (Available 9 a.m. to 5 p.m. CST from Monday to Friday)
icon of address
Address
7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA
icon of social media
Join us with

Subscribe newsletter

Leave a message

* To protect against spam, please pass the CAPTCHA test below.
CAPTCHA verification
© 2007-2024 CUSABIO TECHNOLOGY LLC All rights reserved. 鄂ICP备15011166号-1
webinars: DT3C facilitates antibody internalization X
Place an order now

I. Product details

*
*
*
*

II. Contact details

*
*

III. Ship To

*
*
*
*
*
*
*

IV. Bill To

*
*
*
*
*
*
*
*