Recombinant fragment, corresponding to amino acids 66-176 of Human Ndufs4
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Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 20 kDa.
Use a concentration of 1 µg/ml.
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
Involvement in disease
Defects in NDUFS4 are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease.
Lane 1: Wild-type HAP1 cell lysate (20 µg) Lane 2: Ndufs4knockout HAP1 cell lysate (20 µg) Lane 3: HEK293 cell lysate (20 µg) Lane 4: Hu fetal heart tissue lysate (20 µg) Lanes 1 - 4: Merged signal (red and green). Green - ab55540 observed at 28 kDa. Red - loading control, ab181602, observed at 37 kDa.
ab55540 was shown to recognize Ndufs4 when Ndufs4 knockout samples were used, along with additional cross-reactive bands. Wild-type and Ndufs4 knockout samples were subjected to SDS-PAGE. ab55540 and ab181602 (loading control to GAPDH) at a concentration of 1 µg/ml and a dilution of 1/10000 and incubated overnight at 4°C. Blots were developed with Goat anti-Mouse IgG H&L (IRDye® 800CW) preadsorbed (ab216772) and Goat Anti-Rabbit IgG H&L (IRDye® 680RD) preadsorbed (ab216777) secondary antibodies at 1/10000 dilution for 1 hour at room temperature before imaging.