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.1) Wang, W., et al. Nucleic Acids Res. (2010) In press :
Immunogen
This NDUFV1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 21-50 amino acids from the N-terminal region of human NDUFV1.
Western blot analysis in MDA-MB453 cell line lysates (35ug/lane).
Application Notes
For WB starting dilution is: 1:1000:
Additional Information
Form
Liquid
Storage Instructions
Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.