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Nine novel isovaleryl-CoA dehydrogenase mutations have been found in a Spanish cohort with isovaleric acidemia.
kinetics and ligand binding of isovaleryl-CoA dehydrogenase
A heterogeneous mutation spectrum in the IVD gene was identified in isovaleric acidemia patients in the United Arab Emirates.
A child with isovaleric acidemia was subsequently found to harbour a known missense mutation (c.A1199G [p.Y371C]) and a novel 4-bp duplication (c.1148_1151dupGCTA [p.Y355X]) in the IVD gene. The former may be a founder mutation in the Chinese population.
Replacement of the catalytic glutamate (zeige GRIN1 Proteine) in either short-chain acyl-CoA dehydrogenase (SCAD (zeige Acads Proteine)) or isovaleryl-CoA dehydrogenase (IVD)with glycine resulted in a several-fold reduction in affinity for substrate.
Mutations of isovaleryl-CoA dehydrogenase gene is associated with isovaleric acidemia
Data elucidates the pathway of plant Lys (zeige LYZ Proteine) catabolism and demonstrate that both isovaleryl-CoA dehydrogenase and 2-hydroxyglutarate dehydrogenase (zeige L2HGDH Proteine) act as electron donors to the ubiquinol pool via an ETF (zeige TEAD2 Proteine)/ETFQO (zeige ETFDH Proteine)-mediated route. [IVDH]
Accumulation of both homomethionine and 3-isovaleroyloxypropyl-glucosinolate cosegregates with the high branched chain amino acid phenotype in IVD mutants.
Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.
, isovaleryl-CoA dehydrogenase, mitochondrial
, isovaleryl CoA dehydrogenase
, isovaleryl Coenzyme A dehydrogenase
, isovaleryl coenzyme A dehydrogenase