Mouse Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial (Dbt) ELISA Kit

A Sandwich ELISA from Assay Genie designed for the quantitative measurement of mouse mitochondrial lipoamide acyltransferase, the DBT component of the branched-chain alpha-keto acid dehydrogenase complex. The assay is for research use only and is suited to studies of branched-chain amino acid metabolism, mitochondrial energy production and metabolic disease.

Validated sample types include serum, plasma, tissue homogenates, cell culture supernates and other biological fluids, with specificity for natural and recombinant mouse DBT. The kit reports a detection range of 0.312-20ng/mL and a sensitivity of 0.098ng/mL. Reported linearity values for serum span 89-116% across 1:2 to 1:16 dilutions, EDTA plasma covers 81-122%, and heparin plasma covers 92-110%.

DBT is the E2 lipoamide acyltransferase component of the branched-chain alpha-keto acid dehydrogenase complex, catalysing the transfer of acyl groups to coenzyme A within the mitochondrial matrix. The enzyme forms a 24-polypeptide structural core with octahedral symmetry, and defects in DBT are the cause of maple syrup urine disease type 2, an autosomal recessive disorder.