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.

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.