Source: | Rabbit | Gene Id: | 4723 |
Isotype: | IgG | Swiss Prot: | P49821 |
purity: | Affinity purification |
Background: |
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. |
Reactivity | Human, Mouse, Rat |
Tested applications | WB IF |
Clonality | Polyclonal Antibody |
Calculated MW | 51 kDa |
Recommended Dilutions |
WB 1:500-1:2000
IF 1:50-1:100
|
Immunogen | Recombinant protein of human NDUFV1 |
Storage | Store at -20°C (regular) or -80°C (long term). Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Synonym | UQOR1, CI-51K, CI51KD |
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