Human IVD (Isovaleryl-CoA dehydrogenase) ELISA Kit (HUFI03782)

Isovaleryl-CoA dehydrogenase (IVD) is the mitochondrial matrix enzyme that catalyzes the third step in L-leucine catabolism, converting isovaleryl-CoA into 3-methylcrotonyl-CoA. Deficiency of this enzyme, encoded by the IVD gene on chromosome 15q14-q15, causes isovaleric acidemia, an autosomal recessive organic aciduria presenting in neonates with poor feeding, vomiting, lethargy, metabolic acidosis, and the characteristic "sweety feet" odor during acute decompensation.

The Human IVD (Isovaleryl-CoA dehydrogenase) ELISA Kit from Assay Genie provides an in vitro quantitative assay for measuring IVD protein levels in human clinical specimens. Each kit supplies reagents sufficient for 96 Assays and is configured for human reactivity, supporting research applications in branched-chain amino acid metabolism, carrier-status work-ups for at-risk families, and characterization of patients with suspected mitochondrial fatty-acid and amino-acid oxidation disorders. Results correlate with IVD gene sequencing and with acylcarnitine profiling by tandem mass spectrometry.

The microplate-based workflow is compatible with standard plate readers, and the assay is intended for research use only. Apply standard laboratory practices when handling human-derived specimens, and follow the enclosed package insert for reagent preparation and protocol execution.