NDUFV1 Rabbit Pab

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Leader in Biomolecular Solutions for Life Science NDUFV1 Rabbit pAb Catalog No.: A8014 Basic Information Background Catalog No. The mitochondrial respiratory chain provides energy to cells via oxidative A8014 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 Observed MW of the NADH:ubiquinone oxidoreductase complex I; a large complex with at least 45 40kDa 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 Calculated MW flavin mononucleotide (FMN)- and Fe-S-biding sites. Defects in complex I are a common 49kDa/50kDa cause of mitochondrial dysfunction; a syndrome that occurs in approximately 1 in 10,000 live births. Mitochondrial complex I deficiency is linked to myopathies, Category encephalomyopathies, and neurodegenerative disorders such as Parkinson's disease and Leigh syndrome. Alternative splicing results in multiple transcript variants encoding Primary antibody distinct isoforms. Applications WB, IF Cross-Reactivity Human, Mouse, Rat Recommended Dilutions Immunogen Information WB 1:500 - 1:2000 Gene ID Swiss Prot 4723 P49821 IF 1:50 - 1:100 Immunogen Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human NDUFV1 (NP_009034.2). Synonyms NDUFV1;CI-51K;CI51KD;UQOR1 Contact Product Information www.abclonal.com Source Isotype Purification Rabbit IgG Affinity purification Storage Store at -20℃. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3. Validation Data Western blot analysis of extracts of various cell lines, using NDUFV1 antibody (A8014) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s. Immunofluorescence analysis of A549 cells using NDUFV1 antibody (A8014). Blue: DAPI for nuclear staining. Antibody | Protein | ELISA Kits | Enzyme | NGS | Service For research use only. Not for therapeutic or diagnostic purposes. Please visit http://abclonal.com for a complete listing of recommended products..

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Progressive Encephalopathy and Central Hypoventilation Related to Homozygosity of NDUFV1 Nuclear Gene, a Rare Mitochondrial Disease
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