Anti-NDUFA1 Antibody Picoband® Fluoro550 Conjugated

NDUFA1 (NADH-UBIQUINONE OXIDOREDUCTASE 1 ALPHA SUBCOMPLEX, 1), also called MWFE, B. TAURUS, HOMOLOG OF, encodes a subunit of mitochondrial NADH: ubiquinone oxidoreductase, also known as mitochondrial complex I. The NDUFA1 gene is mapped to chromosome Xq24. The deduced polypeptide sequence of NDUFA1 was found to have an N-terminal hydrophobic domain, likely to be a transmembrane domain, and a C-terminal hydrophilic domain. And the NDUFA1 gene contains 3 exons and spans about 5.0 kb of genomic DNA. Complementation with hamster Ndufa1 cDNA restored the rotenone-sensitive complex I activity of these mutant cells to approximately 100% of the parent cell activity. The findings established that the MWFE polypeptide is absolutely essential for an active complex I in mammals. The NDUFA1 peptide is one of about 31 components of the hydrophobic protein (HP) fraction of complex I which is involved in proton translocation. Thus the NDUFA1 peptide may also participate in that function.

A synthetic peptide corresponding to a sequence at the C-terminus of human NDUFA1, different from the related rat and mouse sequences by one amino acid.

Cancer, Cancer Metabolism, Energy Transfer Pathways, Integration Of Energy, Integration Of Energy Metabolism, Metabolic Signaling Pathway, Metabolic Signaling Pathways, Metabolism, Mitochondria, Mitochondrial, Mitochondrial Markers, Mitochondrial Metabolism, Organelles, Oxidative Phosphorylation, Pathways and Processes, Signal Transduction, Subcellular Markers, Tags & Cell Markers

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.

1. Smeitink J, van den Heuvel L (1999) . Human mitochondrial complex I in health and disease.. Am. J. Hum. Genet. 64 (6) : 1505–10. 2. Au, H. C., Seo, B. B., Matsuno-Yagi, A., Yagi, T., Scheffler, I. E. The NDUFA1 gene product (MWFE protein) is essential for activity of complex I in mammalian mitochondria. Proc. Nat. Acad. Sci. 96: 4354-4359, 1999. 3. Fernandez-Moreira, D., Ugalde, C., Smeets, R., Rodenburg, R. J. T., Lopez-Laso, E., Ruiz-Falco, M. L., Briones, P., Martin, M. A., Smeitink, J. A. M., Arenas, J. X-linked NDUFA1 gene mutations associated with mitochondrial encephalomyopathy. Ann. Neurol. 61: 73-83, 2007.