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FXN Matched Antibody Pair (457) (APMAB-457LY)

This antibody pair set can be used for detecting and quantifying protein level of human FXN.
See all FXN antibodies

Specifications

ApplIcation
Sandwich ELISA
Specificity
Human
Capture Antibody
Rabbit anti-FXN polyclonal antibody, 100 ug
Detection Antibody
Anti-FXN Mouse polyclonal antibody, 20 ug
Dilutions
10 ng/ml-100 ng/ml
Format
Liquid
Storage
Aliquot and store at -20°Cor -80°C. Avoid freeze-thaw cycles.
Introduction
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. [provided by RefSeq, Jul 2016]
Alternative Names
Frataxin; Friedreich Ataxia Protein; FRDA; X25; Frataxin, Mitochondrial; Friedreich Ataxia;
Entrez Gene ID
UniProt ID

Hackett, P. T., Jia, X., Li, L., & Ward, D. M. (2022). Posttranslational regulation of mitochondrial frataxin and identification of compounds that increase frataxin levels in Friedreich’s ataxia. Journal of Biological Chemistry, 298(6).

Medina-Carbonero, M., Sanz-Alcázar, A., Britti, E., Delaspre, F., Cabiscol, E., Ros, J., & Tamarit, J. (2022). Mice harboring the FXN I151F pathological point mutation present decreased frataxin levels, a Friedreich ataxia-like phenotype, and mitochondrial alterations. Cellular and Molecular Life Sciences, 79(2), 74.

Culley, M. K., Zhao, J., Tai, Y. Y., Tang, Y., Perk, D., Negi, V., ... & Chan, S. Y. (2021). Frataxin deficiency promotes endothelial senescence in pulmonary hypertension. The Journal of Clinical Investigation, 131(11).

Doni, D., Rigoni, G., Palumbo, E., Baschiera, E., Peruzzo, R., De Rosa, E., ... & Costantini, P. (2021). The displacement of frataxin from the mitochondrial cristae correlates with abnormal respiratory supercomplexes formation and bioenergetic defects in cells of Friedreich ataxia patients. The FASEB Journal, 35(3), e21362.

Li, J., Li, Y., Wang, J., Gonzalez, T. J., Asokan, A., Napierala, J. S., & Napierala, M. (2020). Defining transcription regulatory elements in the human Frataxin gene: implications for gene therapy. Human gene therapy, 31(15-16), 839-851.

Du, J., Zhou, Y., Li, Y., Xia, J., Chen, Y., Chen, S., ... & Wang, Y. (2020). Identification of Frataxin as a regulator of ferroptosis. Redox Biology, 32, 101483.

Maio, N., Jain, A., & Rouault, T. A. (2020). Mammalian iron–sulfur cluster biogenesis: recent insights into the roles of frataxin, acyl carrier protein and ATPase-mediated transfer to recipient proteins. Current opinion in chemical biology, 55, 34-44.

Patra, S., & Barondeau, D. P. (2019). Mechanism of activation of the human cysteine desulfurase complex by frataxin. Proceedings of the National Academy of Sciences, 116(39), 19421-19430.

Fox, N. G., Yu, X., Feng, X., Bailey, H. J., Martelli, A., Nabhan, J. F., ... & Han, S. (2019). Structure of the human frataxin-bound iron-sulfur cluster assembly complex provides insight into its activation mechanism. Nature communications, 10(1), 2210.

Britti, E., Delaspre, F., Feldman, A., Osborne, M., Greif, H., Tamarit, J., & Ros, J. (2018). Frataxin‐deficient neurons and mice models of Friedreich ataxia are improved by TAT‐MTS cs‐FXN treatment. Journal of cellular and molecular medicine, 22(2), 834-848.

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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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