NADH dehydrogenase [ubiquinone] iron-sulfur protein 2; mitochondrial; Complex I-49kD; CI-49kD; NADH-ubiquinone oxidoreductase 49 kDa subunit; NDUFS2

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This NDUFS2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 286-315 amino acids from the Central region of human NDUFS2.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-NDUFS2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

NDUFS2 is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I). Mammalian mitochondrial complex I is composed of at least 43 different subunits, 7 of which are encoded by the mitochondrial genome, and the rest are the products of nuclear genes. The iron-sulfur protein fraction of complex I is made up of 7 subunits, including this gene product. Complex I catalyzes the NADH oxidation with concomitant ubiquinone reduction and proton ejection out of the mitochondria.

Crown Antibodies; Cancer; Cell Biology; Metabolism; Signal Transduction

ELISA (EIA), Immunohistochemistry (IHC), Western Blot (WB)

WB~~1:1000

Western blot analysis of lysates from A431, Hela cell line, mouse brain and rat brain tissue lysate(from left to right), using NDUFS2 Antibody (Center). MBS9212055 was diluted at 1:1000 at each lane. A goat anti-rabbit IgG H&L(HRP) at 1:10000 dilution was used as the secondary antibody. Lysates at 35ug per lane.

NDUFS2 Antibody (Center) IHC analysis in formalin fixed and paraffin embedded hepatocarcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the NDUFS2 Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.

52546

NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase)

NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial

NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial

CI-49kD??[Similar Products]

NDUS2_HUMAN

The protein encoded by this gene is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I). Mammalian mitochondrial complex I is composed of at least 43 different subunits, 7 of which are encoded by the mitochondrial genome, and the rest are the products of nuclear genes. The iron-sulfur protein fraction of complex I is made up of 7 subunits, including this gene product. Complex I catalyzes the NADH oxidation with concomitant ubiquinone reduction and proton ejection out of the mitochondria. Mutations in this gene are associated with mitochondrial complex I deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Oct 2009]

NDUFS2: Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. Defects in NDUFS2 are a cause of mitochondrial complex I deficiency (MT-C1D). A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, from lethal neonatal disease to *****-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. Belongs to the complex I 49 kDa subunit family.

Protein type: EC 1.6.99.3; Oxidoreductase; Energy Metabolism - oxidative phosphorylation; Mitochondrial; EC 1.6.5.3

Chromosomal Location of Human Ortholog: 1q23

Cellular Component: nucleoplasm; mitochondrion; mitochondrial inner membrane; mitochondrial respiratory chain complex I

Molecular Function: protein binding; electron carrier activity; NADH dehydrogenase (ubiquinone) activity; 4 iron, 4 sulfur cluster binding; metal ion binding; NAD binding; quinone binding; NADH dehydrogenase activity

Biological Process: cellular metabolic process; mitochondrial electron transport, NADH to ubiquinone; response to oxidative stress

Disease: Mitochondrial Complex I Deficiency

Saada, A., et al. Am. J. Hum. Genet. 84(6):718-727(2009) Wang, L., et al. Cancer Epidemiol. Biomarkers Prev. 17(12):3558-3566(2008) Starr, J.M., et al. Mech. Ageing Dev. 129(12):745-751(2008) Ban, M., et al. PLoS ONE 3 (8), E2891 (2008) Triepels, R.H., et al. J. Biol. Chem. 276(12):8892-8897(2001)

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