Western blot analysis of NDUFS3 over-expressed 293 cell line, cotransfected with NDUFS3 Validated Chimera RNAi ( Cat # H00004722-R01V ) (Lane 2) or non-transfected control (Lane 1). Blot probed with NDUFS3 monoclonal antibody (M02), clone 1D6 (Cat # H00004722-M02 ). GAPDH ( 36.1 kDa ) used as specificity and loading control.
This gene encodes one of the iron-sulfur protein (IP) components of mitochondrial NADH:ubiquinone oxidoreductase (complex I). Mutations in this gene are associated with Leigh syndrome resulting from mitochondrial complex I deficiency