NDUFA1 Antibody (3B9-1A1): sc-517006

NDUFA1 Antibody (3B9-1A1) is a mouse monoclonal IgG1 kappa light chain antibody that detects NDUFA1 protein of human origin by western blotting (WB), immunoprecipitation (IP), and enzyme-linked immunosorbent assay (ELISA). Anti-NDUFA1 antibody (3B9-1A1) is available as non-conjugated format. NDUFA1, also known as NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, ZNF183, or MWFE, is a crucial component of Complex I in the oxidative phosphorylation electron transport chain, consisting of approximately 70 amino acids. NDUFA1 is predominantly expressed in skeletal muscle and heart tissues, where NDUFA1 serves as an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase complex. NDUFA1 facilitates the transfer of electrons from NADH to the respiratory chain, a key process in cellular respiration that generates ATP, the energy currency of the cell. NDUFA1 is localized to the matrix side of the mitochondrial inner membrane, and NDUFA1′s structural integrity is essential for the overall stability and activity of Complex I. Defects in the NDUFA1 gene, which maps to human chromosome Xq24, can lead to mitochondrial Complex I deficiency, resulting in a range of serious health issues, including cardiomyopathy, myopathy, liver disease, and neurological disorders.

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NDUFA1 Antibody (3B9-1A1) References:

1. The NDUFA1 gene product (MWFE protein) is essential for activity of complex I in mammalian mitochondria.  |  Au, HC., et al. 1999. Proc Natl Acad Sci U S A. 96: 4354-9. PMID: 10200266
2. Identification of tooth-specific downstream targets of Runx2.  |  Gaikwad, JS., et al. 2001. Gene. 279: 91-7. PMID: 11722849
3. Species-specific and mutant MWFE proteins. Their effect on the assembly of a functional mammalian mitochondrial complex I.  |  Yadava, N., et al. 2002. J Biol Chem. 277: 21221-30. PMID: 11937507
4. SOD2 gene transfer protects against optic neuropathy induced by deficiency of complex I.  |  Qi, X., et al. 2004. Ann Neurol. 56: 182-91. PMID: 15293270
5. X-linked NDUFA1 gene mutations associated with mitochondrial encephalomyopathy.  |  Fernandez-Moreira, D., et al. 2007. Ann Neurol. 61: 73-83. PMID: 17262856
6. A mitochondrial protein compendium elucidates complex I disease biology.  |  Pagliarini, DJ., et al. 2008. Cell. 134: 112-23. PMID: 18614015
7. Isolation, mapping, and genomic structure of an X-linked gene for a subunit of human mitochondrial complex I.  |  Zhuchenko, O., et al. 1996. Genomics. 37: 281-8. PMID: 8938439