Description
This assay is designed for professional diagnostic labs and uses dried blood spot samples on 903® or 226 filter paper. The test involves enzymatic conversion of the branched-chain amino acids (BCAAs) via leucine dehydrogenase, reducing NAD+ to NADH. NADH is then used in a reaction involving tetrazolium salt or resazurin/diaphorase to produce a measurable colorimetric or fluorescent signal whose intensity correlates with BCAA concentration.
This test helps detect various forms of MSUD (classic, intermittent, thiamine‑responsive). If untreated, MSUD leads to accumulation of BCAAs and corresponding keto acids with neurotoxicity, poor feeding, vomiting, and risk of coma. Dietary management and metabolic crisis protocols are critical.
The kit offers different packaging options and is compatible with both manual and automated workflows. It is produced to meet European ISO / CE compliance.
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