Anti-FXN Antibody Picoband®

SKU: A00842-1

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

Applications: WB,IHC,Flow Cytometry,ELISA

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

Gene Name: FXN

Specificity:

Background: This nuclear gene encodes a mitochondrial protein which belongs to the FRATAXIN family. The protein functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA from 8-33 repeats to >90 repeats results in Friedreich ataxia. Alternative splicing results in multiple transcript variants.

Immunogen: E.coli-derived human FXN recombinant protein (Position: Q20-A210). Human FXN shares 79% and 71.4% amino acid (aa) sequence identity with mouse and rat FXN, 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. Adamec, J., Rusnak, F., Owen, W. G., Naylor, S., Benson, L. M., Gacy, A. M., Isaya, G. Iron-dependent self-assembly of recombinant yeast frataxin: implications for Friedreich ataxia. Am. J. Hum. Genet. 67: 549-562, 2000. 2. Adinolfi, S., Trifuoggi, M., Politou, A. S., Martin, S., Pastore, A. A structural approach to understanding the iron-binding properties of phylogenetically different frataxins. Hum. Molec. Genet. 11: 1865-1877, 2002. 3. Anderson, P. A., Kirby, K., Orr, W. C., Hilliker, A. J., Phillips, J. A. Hydrogen peroxide scavenging rescues frataxin deficiency in a Drosophila model of Friedreich's ataxia. Proc. Nat. Acad. Sci. 105: 611-616, 2008.

Uniprot ID: Q16595

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: 14 kDa

Calculated Molecular Weight: 23 kDa

Gene ID: 2395

Protein Name: Frataxin, mitochondrial

Gene Full Name: frataxin

Synonyms: CyaY; d FXN; FA; FARR; frataxin; Frataxin(81 210); Frataxin; mitochondrial; FRDA; Friedreich ataxia protein; FXN; i FXN Frataxin(56 210); m56 FXN Frataxin(78 210); m78 FXN Frataxin mature form; m81 FXN; X25