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Anti-AUH Antibody

品牌：Antibodies

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规格：50µl

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Anti-AUH Antibody

商品详情参考文献相关资料

Overview

Name:	Anti-AUH Antibody See all AUH primary antibodies
Description:	Rabbit polyclonal antibody to AUH.
Applications:	WB, IHC
Dilutions:	WB: 1:500 - 1:2000, IHC: 1:50 - 1:200.
Reactivity:	Human, Mouse, Rat
Immunogen:	Recombinant protein of human AUH.
Protein Length:	339
Host:	Rabbit
Clonality:	Polyclonal
Isotype:	IgG
Conjugate:	Unconjugated
Purification:	Affinity purification.
Product Form:	Liquid
Formulation:	Supplied in Phosphate Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 50% Glycerol.
Storage:	Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

Target

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.
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 (not present in other MGA forms).
Sequence Similarities:	Belongs to the enoyl-CoA hydratase/isomerase family.
Cellular Location:	Mitochondrion.
Database Links:	Entrez Gene: 549?Human Entrez Gene: 11992?Mouse Entrez Gene: 361215?Rat Omim: 600529?Human SwissProt: Q13825?Human SwissProt: Q9JLZ3?Mouse Unigene: 175905?Human Unigene: 252034?Mouse Unigene: 50?Rat
Synonyms:	3 methylglutaconyl CoA hydratase Antibody AU binding protein/enoyl CoA hydratase Antibody AU RNA binding protein/enoyl CoA hydratase Antibody AU RNA binding protein/enoyl Coenzyme A hydratase Antibody AU specific RNA binding Antibody AU specific RNA binding enoyl CoA hydratase Antibody AUH Antibody Methylglutaconyl CoA hydratase Antibody Methylglutaconyl CoA hydratase mitochondrial Antibody MGA Type I Antibody
Information:	Target information shown above is from the UniProt Consortium.