- Catalog number
- PA1661-APC
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- Background
- 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.
- Synonyms
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1;Complex I-MWFE;CI-MWFE;NADH-ubiquinone oxidoreductase MWFE subunit;NDUFA1;
- Gene Name
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1
- UniProt
- O15239
- Host
- Rabbit
- Reactivity
- Human,Mouse,Rat
- Cross Reactivity
- No cross-reactivity with other proteins
- Immunogen
- 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.
- Clonality
- Polyclonal
- Tissue Specificity
- Primarily expressed in heart and skeletal muscle.
- Applications
- IF,IHC,IHC-F,ICC,WB,Flow Cytometry
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- Function
- 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.
- Components
- Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3.
- References & Citations
- 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.
- Storage Conditions
- Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.
- Calculated Molecular Weight
- 8072 MW
- Observed Molecular Weight
- 8 kDa
- Fragment
- Rabbit IgG
- Specificity
- No cross reactivity with other proteins.
- Applications Notes
- Western blot, 0.1-0.5μg/ml, Human, Mouse, Rat<br>
Immunohistochemistry (Paraffin-embedded Section), 2-5μg/ml, Human, Mouse, Rat, By Heat<br>
Immunohistochemistry (Frozen Section), 0.5-1μg/ml, Rat, Human, Mouse<br>
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human<br>
Flow Cytometry(Fixed), 1-3 μg/1x10<sup>6</sup> cells, Human<br>
- Subcellular Location
- Mitochondrion inner membrane; Single-pass membrane protein; Matrix side.
- Sequence Similarities
- Contains 1 death domain.
- Protein Name
- NADH dehydrogenase[ubiquinone] 1 alpha subcomplex subunit 1