The NDUFV1 antibody is a crucial tool for studying the NADH:ubiquinone oxidoreductase core subunit V1 (NDUFV1), a 51 kDa nuclear-encoded component of mitochondrial Complex I (NADH dehydrogenase) in the electron transport chain. As part of the N-module, NDUFV1 plays a pivotal role in transferring electrons from NADH to ubiquinone while contributing to proton translocation. Antibodies targeting NDUFV1 are widely used to investigate Complex I assembly, function, and deficiencies linked to mitochondrial disorders. These antibodies enable detection of NDUFV1 expression levels via Western blotting, immunofluorescence, or immunohistochemistry, helping researchers assess protein localization in tissues or cultured cells. Mutations in the NDUFV1 gene are associated with severe pediatric conditions like Leigh syndrome, encephalopathy, and cardiomyopathy, making these antibodies valuable for diagnostic and mechanistic studies. Commercially available NDUFV1 antibodies are typically raised in rabbits or mice against specific epitopes, with validation in knockout controls to ensure specificity. Recent applications extend to cancer research, as altered Complex I activity influences tumor metabolism. However, researchers must account for tissue-specific isoforms and post-translational modifications that may affect antibody binding. Proper validation using mitochondrial fractions and genetic models remains essential for reliable data interpretation in both basic and clinical research contexts.