CI-49

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

Polyclonal

Rabbit

The antibody detects endogenous levels of total NDUFS2 protein.

Antigen affinity purification.

Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.

2.1 mg/ml (lot specific)

Store at -20 degree C

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.

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.

Total protein Ab

Western Blot (WB), Immunohistochemistry (IHC)

Western blotting: 1:200-1:1000
Immunohistochemistry: 1:50-1:200

Gel: 8%SDS-PAGE Lysates (from left to right): Mouse heart tissue Amount of lysate: 40ug per lane Primary antibody: 1/250 dilution Secondary antibody dilution: 1/8000 Exposure time: 5 minutes

Immunohistochemical analysis of paraffin-embedded Human thyroid cancer tissue using at dilution 1/35.

51,852 Da

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: Energy Metabolism - oxidative phosphorylation; Oxidoreductase; Mitochondrial; EC 1.6.99.3; 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; NADH dehydrogenase (ubiquinone) activity; electron carrier activity; 4 iron, 4 sulfur cluster binding; metal ion binding; quinone binding; NAD binding; NADH dehydrogenase activity

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

Disease: Mitochondrial Complex I Deficiency

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