The mitochondrial respiratory chain provides energy to cells via oxidative phosphorylation and consists of four membrane-bound electron-transporting protein complexes (I-IV) and an ATP synthase (complex V). This gene encodes a 51 kDa subunit of the NADH:ubiquinone oxidoreductase complex I; a large complex with at least 45 nuclear and mitochondrial encoded subunits that liberates electrons from NADH and channels them to ubiquinone. This subunit carries the NADH-binding site as well as flavin mononucleotide (FMN)- and Fe-S-biding sites. Defects in complex I are a common cause of mitochondrial dysfunction; a syndrome that occurs in approximately 1 in 10,000 live births. Mitochondrial complex I deficiency is linked to myopathies, encephalomyopathies, and neurodegenerative disorders such as Parkinson's disease and Leigh syndrome. Alternative splicing results in multiple transcript variants encoding distinct isoforms.
Dilutions
WB 1:500 - 1:2000 IF 1:50 - 1:100
Format
Calculated MW
49kDa/50kDa
Observed MW
40kDa
Immunogen
Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human NDUFV1 (NP_009034.2).
Modification
Unmodified
Storage
-20°C
Images
Western blot analysis of extracts of various cell lines, using NDUFV1 antibody (144-08014) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 30s.
Immunofluorescence analysis of A549 cells using NDUFV1 antibody (144-08014). Blue: DAPI for nuclear staining.
Expiration:
12 months from the date of shipment when stored properly.
