Code | CSB-RA224121A0HU |
Size | US$210 |
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Application | Recommended Dilution |
---|---|
WB | 1:500-1:5000 |
IHC | 1:50-1:200 |
NDUFS3 is a subunit of the NADH: ubiquinone oxidoreductase ETC complex I, which catalyzes the initial stage of electron transfer from NADH to a noncovalently bound flavin mononucleotide and subsequently to the terminal acceptor ubiquinone via a succession of iron-sulfur clusters. NDUFS3 is recruited to the inner mitochondrial membrane to form an early assembly intermediate with NDUFS2 and plays a critical role in the correct assembly of complex I. It starts the complex I assembly process in the mitochondrial matrix. Complex I function is disrupted by NDUFS3 cleavage, resulting in superoxide-dependent but MOMP-independent cell death. NDUFS3 gene missense mutations cause severe encephalomyopathy, including Leigh syndrome (LS).
CUSABIO designed the vector clones for the expression of a recombinant NDUFS3 antibody in mammalian cells. The vector clones were obtained by inserting the NDUFS3 antibody heavy and light chains into the plasma vectors. The recombinant NDUFS3 antibody was purified from the culture medium through Affinity-chromatography. It can be used to detect NDUFS3 protein from Human in the ELISA, WB, IHC.There are currently no reviews for this product.