Anti-NDUFS4 Antibody Picoband®

SKU: A03608-3

Size: 100 μg

Storage: At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,IHC,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Immunohistochemistry (Paraffin-embedded Section), 2-5 μg/ml, Human, Mouse, Rat
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml

Gene Name: NDUFS4

Specificity:

Background: This gene encodes an nuclear-encoded accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I, or NADH:ubiquinone oxidoreductase). Complex I removes electrons from NADH and passes them to the electron acceptor ubiquinone. Mutations in this gene can cause mitochondrial complex I deficiencies such as Leigh syndrome. Alternative splicing results in multiple transcript variants.

Immunogen: E.coli-derived human NDUFS4 recombinant protein (Position: R29-K175). Human NDUFS4shares 91.8% and 90.5% amino acid (aa) sequence identity with mouse and rat NDUFS4, respectively.

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Anderson, S. L., Chung, W. K., Frezzo, J., Papp, J. C., Ekstein, J., DiMauro, S., Rubin, B. Y. A novel mutation in NDUFS4 causes Leigh syndrome in an Ashkenazi Jewish family. J. Inherit. Metab. Dis. 31: S461-S467, 2008. 2. Assereto, S., Robbiano, A., Di Rocco, M., Rossi, A., Cassandrini, D., Panicucci, C., Brigati, G., Biancheri, R., Bruno, C., Minetti, C., Trucks, H., Sander, T., Zara, F., Gazzerro, E. Functional characterization of the c.462delA mutation in the NDUFS4 subunit gene of mitochondrial complex I. (Letter) Clin. Genet. 86: 99-101, 2014. 3. Benit, P., Steffann, J., Lebon, S., Chretien, D., Kadhom, N., de Lonlay, P., Goldenberg, A., Dumez, Y., Dommergues, M., Rustin, P., Munnich, A., Rotig, A. Genotyping microsatellite DNA markers at putative disease loci in inbred/multiplex families with respiratory chain complex I deficiency allows rapid identification of a novel nonsense mutation (IVS1nt -1) in the NDUFS4 gene in Leigh syndrome. Hum. Genet. 112: 563-566, 2003.

Uniprot ID: O43181

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 20 kDa

Calculated Molecular Weight: 20 kDa

Gene ID: 4724

Protein Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial

Gene Full Name: NADH:ubiquinone oxidoreductase subunit S4

Synonyms: AQDQ; CI 18 kDa; CI AQDQ; Complex I 18 kDa; Complex I AQDQ; NDUFS4