84-2222-01　［取扱停止］NDUFV1 (NADH Dehydrogenase [Ubiquinone] Flavoprotein 1, Mitochondrial, NADH-Ubiquinone Oxidoreductase 51kD Subunit, UQOR1, CI-51K, CI51KD) 200ul　212233

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 51kD 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.
• Other applications not tested.
• Recommended Dilution:Western Blot: 1:200-1:2000Optimal dilutions to be determined by the researcher.
• 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 after receipt.
• For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.