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.
SDS-PAGE - AUH protein (ab101821)
15% SDS-PAGE analysis of 3µg ab101821.
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