84-1306-36　NDUFV1, NT (NDUFV1, UQOR1, NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial, Complex I-51kD, NADH dehydrogenase flavoprotein 1, NADH-ubiquinone oxidoreductase 51kD subunit) 200ul　038872

特徴

• 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.
• Applications:Suitable for use in Western Blot, ELISA
• Recommended Dilution:ELISA: 1:1,000Western Blot: 1:100-500
• Storage and Stability:May be stored at 4°C for short-term only.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• Aliquots are stable for 12 months.
• For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.