Human HIBCH ELISA Kit (HUFI07808)

Quantify 3-hydroxyisobutyryl-CoA hydrolase with this in vitro research immunoassay from Assay Genie, formatted as a 96 Assays kit for laboratory use. The enzyme HIBCH localizes to mitochondria and participates in the final step of valine catabolism, converting 3-hydroxyisobutyryl-CoA into 3-hydroxyisobutyrate. Research interest in this target centers on HIBCH deficiency, an autosomal recessive neurometabolic disorder linked to Leigh-like encephalopathy, psychomotor regression, and elevated C2-carnitine in newborn screening programs. Each kit contains pre-coated microplate wells, biotinylated detection antibody, standards, and streptavidin-HRP conjugate for chromogenic readout, and is compatible with serum-derived and plasma-derived specimens. Reconstituted reagents should be stored refrigerated and protected from light, with working solutions prepared fresh before each run. Standard curves generated from serial dilutions support reliable quantitation across the assay range. Researchers studying mitochondrial beta-oxidation disorders, branched-chain amino acid metabolism, or pediatric neurodegeneration workflows can integrate this kit into existing ELISA platforms without specialized equipment.