Anti-AUH antibody (AA39771)

$187.00


Alternative names Methylglutaconyl-CoA hydratase, mitochondrial, EC 4.2.1.18, AU-specific RNA-binding enoyl-CoA hydratase, AU-binding protein/enoyl-CoA hydratase, AUH, AUHM_HUMAN, Q13825, 549
Source 339
Species Reactivity Human, Mouse, Rat
Applications IHC, WB
Host Rabbit
Class Polyclonal
Conjugated Unconjugated


Description

Product name Anti-AUH antibody (AA39771)
Catalog number AA39771
Brand family Ango
Product type Primary Antibody
Antibody Alternative Names Methylglutaconyl-CoA hydratase, mitochondrial antibody, EC 4.2.1.18 antibody, AU-specific RNA-binding enoyl-CoA hydratase antibody, AU-binding protein/enoyl-CoA hydratase antibody, AUH antibody, AUHM_HUMAN antibody


Biological Information

Immunogen Recombinant protein of human AUH
Concentration 1 mg/ml
Isotype IgG
Application Notes WB 1:500 - 1:2000 IHC 1:50 - 1:200
Purification method Purified by affinity chromatography


Target

Target Name AUH
SwissProt ID Link Q13825
Function 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.
Subcellular Location Mitochondrion.
Involvement in Disease 3-methylglutaconic aciduria 1: 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 . The disease is caused by mutations affecting the gene represented in this entry.
Subunit Structure Homohexamer.
Sequence Similarities Belongs to the enoyl-CoA hydratase/isomerase family.


Poduct Presentation

Form Liquid
Preservative 0.02% (w/v) sodium azide
Storage buffer PBS with 50% glycerol, pH 7.3


Storage and Shipping Information

Storage Store at -20 or -80 C. Avoid multiple freeze/thaw cycles.