The NDUFB11 antibody is a tool used to detect the NDUFB11 protein, a subunit of mitochondrial Complex I (NADH:ubiquinone oxidoreductase) in the electron transport chain. Complex I plays a critical role in cellular energy production by facilitating NADH oxidation and proton translocation across the mitochondrial inner membrane, driving ATP synthesis. NDUFB11. encoded by the nuclear *NDUFB11* gene (located on the X chromosome), is essential for Complex I assembly and stability. Mutations in this gene are linked to mitochondrial disorders, such as mitochondrial encephalopathy, cardiomyopathy, and isolated Complex I deficiency, often presenting with neurological or metabolic impairments.
The antibody is widely employed in research to study mitochondrial dysfunction in diseases like Leigh syndrome, neurodegenerative disorders, and cancers, where altered energy metabolism is a hallmark. It aids in detecting protein expression levels via Western blotting, immunofluorescence, or immunohistochemistry, enabling insights into tissue-specific Complex I deficiencies. Additionally, it serves as a diagnostic tool in genetic screenings and mechanistic studies exploring the role of oxidative phosphorylation defects in cellular pathologies.
As mitochondrial research advances, the NDUFB11 antibody remains pivotal for understanding the molecular basis of Complex I-related diseases and developing targeted therapeutic strategies. Its specificity and reliability make it valuable for both basic and clinical investigations into mitochondrial biology.