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Western Blot (WB) (All lanes: Methylglutaconyl-CoA hydratase, mitochondrial antibody at 6ug/mlLane 1:Rat brain tissueLane 2:Rat kidney tissueSecondaryGoat polyclonal to rabbit at 1/10000 dilutionPredicted band size: 36,33 kDaObserved band size: 36 kDa)

Rabbit anti-Human, Rat Methylglutaconyl-CoA hydratase Polyclonal Antibody | anti-AUH antibody

Rabbit anti-human Methylglutaconyl-CoA hydratase, mitochondrial polyclonal Antibody(AUH)

Reactivity
Human, Rat
Applications
ELISA, Western Blot, Immunohistochemistry
Purity
Antigen Affinity Purified
Synonyms
Methylglutaconyl-CoA hydratase; Polyclonal Antibody; Rabbit anti-human Methylglutaconyl-CoA hydratase; mitochondrial polyclonal Antibody(AUH); AU-specific RNA-binding enoyl-CoA hydratase; AU-binding protein/enoyl-CoA hydratase; AUH; anti-AUH antibody
Ordering
For Research Use Only!
Host
Rabbit
Reactivity
Human, Rat
Clonality
Polyclonal
Isotype
IgG
Purity/Purification
Antigen Affinity Purified
Sequence Length
310
Applicable Applications for anti-AUH antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
Application Notes
Recommended dilution: WB:1:500-2000
IHC:1:20-1:200
Storage Buffer
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Conjugate
Non-conjugated
Immunogen
Recombinant human Methylglutaconyl-CoA hydratase, mitochondrial protein (68-339aa)
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-82518 / sc-82521
Preparation and Storage
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.

Western Blot (WB)

(All lanes: Methylglutaconyl-CoA hydratase, mitochondrial antibody at 6ug/mlLane 1:Rat brain tissueLane 2:Rat kidney tissueSecondaryGoat polyclonal to rabbit at 1/10000 dilutionPredicted band size: 36,33 kDaObserved band size: 36 kDa)

Western Blot (WB) (All lanes: Methylglutaconyl-CoA hydratase, mitochondrial antibody at 6ug/mlLane 1:Rat brain tissueLane 2:Rat kidney tissueSecondaryGoat polyclonal to rabbit at 1/10000 dilutionPredicted band size: 36,33 kDaObserved band size: 36 kDa)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human tonsil using MBS1494224 at dilution 1:100)

Immunohistochemistry (IHC) (Immunohistochemistry of paraffin-embedded human tonsil using MBS1494224 at dilution 1:100)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human kidney using MBS1494224 at dilution 1:100)

Immunohistochemistry (IHC) (Immunohistochemistry of paraffin-embedded human kidney using MBS1494224 at dilution 1:100)
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.

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
549
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Accession #
Molecular Weight
32,508 Da
NCBI Official Full Name
methylglutaconyl-CoA hydratase, mitochondrial isoform 2
NCBI Official Synonym Full Names
AU RNA binding protein/enoyl-CoA hydratase
NCBI Official Symbol
AUH
NCBI Protein Information
methylglutaconyl-CoA hydratase, mitochondrial
UniProt Protein Name
Methylglutaconyl-CoA hydratase, mitochondrial
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: Lyase; RNA-binding; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 4.2.1.18

Chromosomal Location of Human Ortholog: 9q22.31

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function: enoyl-CoA hydratase activity; methylglutaconyl-CoA hydratase activity; mRNA 3'-UTR binding

Biological Process: branched chain family amino acid catabolic process; leucine catabolic process

Disease: 3-methylglutaconic Aciduria, Type I

Research Articles on AUH

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Product Notes

The AUH auh (Catalog #AAA1494224) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Rabbit anti-human Methylglutaconyl-CoA hydratase, mitochondrial polyclonal Antibody(AUH) reacts with Human, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's Methylglutaconyl-CoA hydratase can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC). Recommended dilution: WB:1:500-2000 IHC:1:20-1:200. Researchers should empirically determine the suitability of the AUH auh for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Methylglutaconyl-CoA hydratase, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.

Precautions

All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.

Disclaimer

Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.

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