Anti-NDUFS3 Antibody Picoband® PE Vervoeging

This gene encodes one of the iron-sulfur protein (IP) components of mitochondrial NADH:ubiquinone oxidoreductase (complex I) . Mutations in this gene are associated with Leigh syndrome resulting from mitochondrial complex I deficiency.

NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial; EC 1.6.99.3; EC 7.1.1.2; Complex I-30kD; CI-30kD; NADH-ubiquinone oxidoreductase 30 kDa subunit

NDUFS3

4722

O75489

Human, Mouse, Rat

No cross-reactivity with other proteins

E.coli-derived human NDUFS3 recombinant protein (Position: R26-K264) . Human NDUFS3 shares 90.4% amino acid (aa) sequence identity with mouse NDUFS3.

Flow Cytometry

Immunogen affinity purified.

Liquid

1. Benit, P., Slama, A., Cartault, F., Giurgea, I., Chretien, D., Lebon, S., Marsac, C., Munnich, A., Rotig, A., Rustin, P. Mutant NDUFS3 subunit of mitochondrial complex I causes Leigh syndrome. J. Med. Genet. 41: 14-17, 2004. 2. Emahazion, T., Beskow, A., Gyllensten, U., Brookes, A. J. Intron based radiation hybrid mapping of 15 complex I genes of the human electron transport chain. Cytogenet. Cell Genet. 82: 115-119, 1998. 3. Haack, T. B., Haberberger, B., Frisch, E.-M., Wieland, T., Iuso, A., Gorza, M., Strecker, V., Graf, E., Mayr, J. A., Herberg, U., Hennermann, J. B., Klopstock, T., and 16 others. Molecular diagnosis in mitochondrial complex I deficiency using exome sequencing. J. Med. Genet. 49: 277-283, 2012.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

30 kDa

6

NADH:ubiquinone oxidoreductase core subunit S3

NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.