Metabolism Antibodies 2

Anti-AUH Antibody (CAB7424)

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SKU:
CAB7424
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism

Description

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Antibody Name:Anti-AUH Antibody
Antibody SKU:CAB7424
Antibody Size:20uL, 50uL, 100uL
Application:WB IHC
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 68-339 of human AUH (NP_001689.1).
Application:WB IHC
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:A-549, SW480, HepG2, Mouse brain, Mouse heart, Mouse kidney, Rat brain, Rat heart, Rat kidney
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 68-339 of human AUH (NP_001689.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:SSEM KTED ELRV RHLE EENR GIVV LGIN RAYG KNSL SKNL IKML SKAV DALK SDKK VRTI IIRS EVPG IFCA GADL KERA KMSS SEVG PFVS KIRA VIND IANL PVPT IAAI DGLA LGGG LELA LACD IRVA ASSA KMGL VETK LAII PGGG GTQR LPRA IGMS LAKE LIFS ARVL DGKE AKAV GLIS HVLE QNQE GDAA YRKA LDLA REFL PQGP VAMR VAKL AINQ GMEV DLVT GLAI EEAC YAQT IPTK DRLE GLLA FKEK RPPR YKGE
Gene ID:549
Uniprot:Q13825
Cellular Location:Mitochondrion
Calculated MW:32kDa/35kDa
Observed MW:30kDa
Synonyms:AUH
Background: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.
UniProt Protein Function: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.
UniProt Protein Details:

Protein type:Mitochondrial; Lyase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 4.2.1.18; RNA-binding

Chromosomal Location of Human Ortholog: 9q22.31

Cellular Component: mitochondrion; mitochondrial matrix

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

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

Disease: 3-methylglutaconic Aciduria, Type I

NCBI Summary: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 Code:Q13825
NCBI GenInfo Identifier:37076898
NCBI Gene ID:549
NCBI Accession:Q13825.1
UniProt Related Accession:Q13825
Molecular Weight:37kDa
NCBI Full Name:Methylglutaconyl-CoA hydratase, mitochondrial
NCBI Synonym Full Names:AU RNA binding methylglutaconyl-CoA hydratase
NCBI Official Symbol:AUH  
NCBI Protein Information:methylglutaconyl-CoA hydratase, mitochondrial
UniProt Protein Name:Methylglutaconyl-CoA hydratase, mitochondrial
UniProt Synonym Protein Names:AU-specific RNA-binding enoyl-CoA hydratase; AU-binding protein/enoyl-CoA hydratase
Protein Family:Methylglutaconyl-CoA hydratase
UniProt Gene Name:AUH  
UniProt Entry Name:AUHM_HUMAN
Anti-AUH Antibody (CAB7424)Western blot analysis of extracts of various cell lines, using AUH antibody (CAB7424) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 60s.
Anti-AUH Antibody (CAB7424)Immunohistochemistry of paraffin-embedded rat kidney using AUH antibody (CAB7424) at dilution of 1:100 (40x lens).
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