NDUFAF1抗体—艾普蒂

The NDUFAF1 antibody is a crucial tool in studying mitochondrial complex I (NADH:ubiquinone oxidoreductase) deficiencies and related disorders. NDUFAF1 (NADH Dehydrogenase [Ubiquinone] 1 Alpha Subcomplex Assembly Factor 1) is a nuclear-encoded protein essential for the assembly and stability of mitochondrial complex I, the largest enzyme in the electron transport chain. Mutations in the NDUFAF1 gene disrupt oxidative phosphorylation, leading to mitochondrial diseases such as Leigh syndrome, cardiomyopathy, and encephalopathy. Researchers use NDUFAF1 antibodies to detect protein expression levels, assess subcellular localization via immunofluorescence or immunohistochemistry, and investigate disease mechanisms in cell or animal models. These antibodies are also employed in diagnostics to identify patients with complex I deficiencies caused by NDUFAF1 mutations. Commercially available as monoclonal or polyclonal variants, they are validated for specificity across techniques like Western blotting and flow cytometry. Studies utilizing NDUFAF1 antibodies have advanced understanding of mitochondrial dynamics, energy metabolism defects, and potential therapeutic targets for rare genetic disorders. Proper validation with knockout controls remains critical due to cross-reactivity risks in mitochondrial protein studies.