Anti-NDUFA1 / NADH dehydrogenase ubiquinone 1 alpha subcomplex subunit 1

Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml. NDUFA1 antibody detects NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1, a core accessory protein of mitochondrial Complex I that contributes to oxidative phosphorylation and energy metabolism. Encoded by the NDUFA1 gene on the X chromosome (Xp11.3), this protein is one of approximately 45 subunits forming NADH:ubiquinone oxidoreductase, the first enzyme in the mitochondrial electron transport chain. NDUFA1 helps anchor hydrophobic membrane components of Complex I to its hydrophilic catalytic core, maintaining structural stability and supporting efficient electron transfer from NADH to ubiquinone.NDUFA1 is a small, highly conserved protein localized to the mitochondrial inner membrane. It associates with other accessory subunits of the alpha subcomplex, including NDUFA2 and NDUFA3, and is essential for the assembly and functional integrity of Complex I. By stabilizing the membrane domain, NDUFA1 ensures proper coupling of proton translocation with electron transfer, thereby contributing to the mitochondrial proton gradient and ATP synthesis. Mutations in NDUFA1 can impair Complex I activity, leading to mitochondrial encephalomyopathy, lactic acidosis, and neurodegenerative diseases.The NDUFA1 antibody is widely used in mitochondrial biology, metabolism, and neurodegeneration research to study electron transport chain organization and oxidative phosphorylation. Western blot analysis identifies a 7 kilodalton band corresponding to NDUFA1, while immunofluorescence reveals mitochondrial localization colocalizing with markers such as TOM20 and COX IV. This antibody allows characterization of mitochondrial respiratory complexes under physiological and disease conditions.Defects in Complex I assembly caused by NDUFA1 dysfunction contribute to reduced ATP production and excessive reactive oxygen species generation, triggering oxidative stress and apoptosis. The NDUFA1 antibody provides a key tool for investigating mitochondrial respiratory disorders, metabolic adaptation, and redox regulation. NSJ Bioreagents supplies this antibody validated for its applications, ensuring precise and reproducible detection of NDUFA1 in mitochondrial research.