Anti-NDUFS3 Antibody Picoband® Biotin Conjugated
Product Specifications
Background
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.
Synonyms
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
Gene Name
NDUFS3
Gene ID
4722
UniProt
O75489
Host
Rabbit
Reactivity
Human, Mouse, Rat
Cross Reactivity
No cross-reactivity with other proteins
Immunogen
E.coli-derived human NDUFS3 recombinant protein (Position: R26-K264) . Human NDUFS3 shares 90.4% amino acid (aa) sequence identity with mouse NDUFS3.
Clonality
Polyclonal
Applications
WB, IHC, ELISA
Purification
Immunogen affinity purified.
Form
Liquid
References & Citations
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.
Storage Conditions
At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing.
Calculated Molecular Weight
30 kDa
Gene Name Synonym
NADH:ubiquinone oxidoreductase core subunit S3
Protein Name
NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial
Isotype
Rabbit IgG
Contents
Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.
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