Name: Anti-NDUFV1 antibody
Price: 297.00 USD

Overview
- 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.
  
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Properties
- Host
  
  rabbit
  
  Antibody Type
  
  polyclonal
  
  Isotype
  
  IgG
  
  Reactivity
  
  mouse; rat
  
  Immunogen
  
  Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human NDUFV1 (NP_009034. 2).
  
  Purification
  
  Affinity purification
  
  Conjugate
  
  Unconjugated
  
  Formulation
  
  PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  
  Storage
  
  Store at -20°C and avoid freeze-thaw cycles.
  
  Regulatory Status
  
  RUO
  
  Antigen
  
  Nadh Dehydrogenase Ubiquinone Flavoprotein 1-Mitochondrial; Complex I-51kd; Ci-51kd; Nadh Dehydrogenase Flavoprotein 1; Nadh-Ubiquinone Oxidoreductase 51 Kda Subunit (Gene ID:4723; UniProt:P49821)
  
  * For research use only