IVD Human
Description
The IVD is fused to a 20 amino acid his tag at N-terminus and purified by conventional chromatography.
Product Specs
Product Science Overview
Isovaleryl Coenzyme A Dehydrogenase is encoded by the IVD gene located on chromosome 15 in humans . The enzyme is a member of the acyl-CoA dehydrogenase family, which is characterized by its ability to catalyze the dehydrogenation of acyl-CoA derivatives. The enzyme’s active site binds flavin adenine dinucleotide (FAD) as a cofactor, which is essential for its catalytic activity .
The primary function of IVD is to facilitate the breakdown of leucine, an essential amino acid, by catalyzing the conversion of isovaleryl-CoA to 3-methylcrotonyl-CoA. This reaction is a critical step in the leucine catabolic pathway, which ultimately leads to the production of acetyl-CoA and acetoacetate, molecules that can be utilized for energy production .
A deficiency in IVD activity due to mutations in the IVD gene results in a metabolic disorder known as isovaleric acidemia . This condition is characterized by the accumulation of isovaleric acid, a toxic compound that can lead to severe metabolic disturbances. Symptoms of isovaleric acidemia can range from mild to life-threatening and may include vomiting, lethargy, seizures, and a distinctive “sweaty feet” odor .
Isovaleric acidemia can present in two major clinical forms: an acute neonatal form and a chronic intermittent form. The acute form typically manifests shortly after birth with severe metabolic crises, while the chronic form presents later in life with intermittent episodes of metabolic decompensation .
The recombinant form of Isovaleryl Coenzyme A Dehydrogenase is produced using genetic engineering techniques. The IVD gene is cloned into an expression vector, which is then introduced into a suitable host organism, such as Escherichia coli or yeast. The host cells are cultured under conditions that promote the expression of the recombinant enzyme, which is subsequently purified using chromatographic techniques .
Recombinant IVD is used in various research applications, including studies on enzyme kinetics, structure-function relationships, and the development of therapeutic strategies for isovaleric acidemia. The availability of human recombinant IVD allows for detailed biochemical and biophysical analyses, which are essential for understanding the enzyme’s function and its role in metabolic disorders .
