13 products were found matching "K00253"!

Isovaleroylglycine is a compound found in the urine of patients with isovaleric acidemia, a disorder created by defective leucine metabolism due to a deficiency in the isovaleryl coenzyme A dehydrogenase. It has been used as a diagnostic tool in the diagnosis of various acidemias and mitochondrial fatty acid...

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...

Isovaleryl-DL-carnitine-d3 is intended for use as an internal standard for the quantification of isovaleryl-DL-carnitine by GC- or LC-MS. Isovaleryl-DL-carnitine is a derivative of carnitine (Cay-21489, Cay-16749). Increased levels of isovaleryl carnitine are associated with isovaleric acidemia, an inborn error of...

IVD(Isovaleryl CoA dehydrogenase,mitochondrial) is a member of the acylCoA dehydrogenase family and is involved in the catabolism of leucine. In size,IVD precursor and mature proteins produced by class I mutants are indistinguishable from their normal counterparts. Class II,III,and IV mutants make IVD precursor...

WB 1:200 - 1:2000.

Protein function: Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl- CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway (PubMed:7640268). To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as...

Isovaleryl-DL-carnitine-d9 is intended for use as an internal standard for the quantification of isovaleryl-DL-carnitine by GC- or LC-MS. Isovaleryl-DL-carnitine is a derivative of carnitine (Cay-21489, Cay-16749). Increased levels of isovaleryl carnitine are associated with isovaleric acidemia, an inborn error of...

Protein function: Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl- CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway (PubMed:7640268). To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as...

IVD (Isovaleryl Coenzyme A dehydrogenase) is a mitochondrial matrix enzyme that belongs to the acyl-CoA dehydrogenase family. IVD is a homotetrameric flavoenzyme which catalyzes the conversion of isovaleryl-CoA to 3-methylcrotonyl-CoA. Defects of the IVD gene lead to ineffective isoforms that are the underlying...

Human IVD (Isovaleryl-CoA dehydrogenase) ELISA Kit from Assay Genie is a pre-coated immunoassay with a high sensitivity and broad range and has been designed to measure Human IVD (Isovaleryl-CoA dehydrogenase) in serum, plasma & cell culture supernatant samples. The Human IVD (Isovaleryl-CoA dehydrogenase) ELISA Kit...

Protein function: Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl- CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway (PubMed:7640268). To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as...