Anti-AUH monoclonal antibody, clone FQS22198(C)

Antibody Isotype

IgG

Clone

FQS22198(C)

Species Reactivity

Mouse; Rat; Human

Immunogen

Synthetic peptide, corresponding to residues in Human AUH isoform 2 (UniProt ID: Q13825-2).

Conjugate

Application Notes

WB: 1/1000 - 1/10000; IHC-P: 1/50 - 1/100; IP: 1/10 - 1/100;

Alternative Names

AUH; AU RNA binding protein/enoyl-CoA hydratase; AU RNA binding protein/enoyl Coenzyme A hydratase , AU RNA binding protein/enoyl Coenzyme A hydratase; methylglutaconyl-CoA hydratase, mitochondrial; 3-methylglutaconyl-CoA hydratase; AU-binding protein/En

Entrez Gene ID

UniProt ID

Antigen Description

AUH (3-methylglutaconyl-CoA hydratase) catalyzes the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA and has very low enoyl-CoA hydratase activity. Deletion or mutation of the AUH gene causes the metabolic disease 3-methylglutaconic aciduria type I (MGA1). MGA type I is characterized by an abnormal organic acid profile in which there is excessive urinary excretion of 3-methylglutaconic acid, 3-methylglutaric acid and 3-hydroxyisovaleric acid. AUH is also an RNA-binding protein that binds in vitro to clustered 5-AUUUA-3 motifs.

Pathway

Branched-chain amino acid catabolism, organism-specific biosystem; Leucine degradation, leucine => acetoacetate + acetyl-CoA, organism-specific biosystem; Leucine degradation, leucine => acetoacetate + acetyl-CoA, conserved biosystem; Metabolic pathways, organism-specific biosystem; Metabolism, organism-specific biosystem;