Human NDUFB11 ELISA Kit (HUFI07539)

The Assay Genie NDUFB11 immunoassay targets the NADH:ubiquinone oxidoreductase subunit B11 polypeptide, a small iron-sulfur binding component of the matrix-facing arm of mitochondrial Complex I encoded on chromosome Xp11.23. Quantitative readouts support studies of Complex I assembly in patient-derived fibroblasts and lymphoblastoid lines from cohorts with suspected mitochondrial cytopathies, including the X-linked linear skin defects with multiple congenital anomalies (LSDMCA1) spectrum. The protocol accommodates liquid biopsy matrices from translational studies of electron transport chain biomarkers, with intra-batch CV reported below 10 percent across the working range. Reconstituted standards remain stable for short-term refrigerated use, while the microplate, antibody cocktail, and chromogenic substrate are shipped together on insulated cold packs to preserve enzymatic conjugate activity. Researchers studying NADH dehydrogenase stoichiometry, mitochondrial supercomplex formation, or rare X-linked metabolic disease can integrate the kit into existing ELISA workflows alongside matched Assay Genie reagents. Working conjugate and wash buffer should be brought to room temperature before use, and the TMB substrate protected from light until plate development.