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- Antigen structure
- References [24]
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- Product number
- 14147-1-AP - Provider product page
- Provider
- Proteintech Group
- Proper citation
- Proteintech Cat#14147-1-AP, RRID:AB_2231876
- Product name
- FXN antibody
- Antibody type
- Polyclonal
- Description
- FXN antibody (Cat. #14147-1-AP) is a rabbit polyclonal antibody that shows reactivity with human, mouse, rat and has been validated for the following applications: WB,ELISA.
- Reactivity
- Human, Mouse, Rat
- Host
- Rabbit
- Conjugate
- Unconjugated
- Isotype
- IgG
- Vial size
- 20ul, 150ul
Submitted references PLZ4@SeD-induced ferroptosis sensitizes bladder cancer to chemotherapy and immunotherapy.
Antisense oligonucleotide therapy for patients with Friedreich's ataxia carrying the c.165+5G>C splicing mutation.
Analysis and verification of potential ferroptosis-related diagnostic markers in the early stage of metabolic dysfunction-associated steatohepatitis.
Impaired Iron-Sulfur Cluster Synthesis Induces Mitochondrial PARthanatos in Diabetic Cardiomyopathy.
Gene editing improves endoplasmic reticulum-mitochondrial contacts and unfolded protein response in Friedreich's ataxia iPSC-derived neurons.
Reactive oxygen species control protein degradation at the mitochondrial import gate.
Design and validation of cell-based potency assays for frataxin supplementation treatments.
mTORC1 Signaling Inhibition Modulates Mitochondrial Function in Frataxin Deficiency.
FXN targeting induces cell death in ovarian cancer stem-like cells through PRDX3-Mediated oxidative stress.
Comparative multi-omic analyses of cardiac mitochondrial stress in three mouse models of frataxin deficiency.
Proteomic Study on the Mechanism of Arsenic Neurotoxicity in the Rat Cerebral Cortex and the Protective Mechanism of Dictyophora Polysaccharides against Arsenic Neurotoxicity.
Neurobehavioral deficits of mice expressing a low level of G127V mutant frataxin.
Human frataxin, the Friedreich ataxia deficient protein, interacts with mitochondrial respiratory chain.
Iron-frataxin involved in the protective effect of quercetin against alcohol-induced liver mitochondrial dysfunction.
Selected Histone Deacetylase Inhibitors Reverse the Frataxin Transcriptional Defect in a Novel Friedreich's Ataxia Induced Pluripotent Stem Cell-Derived Neuronal Reporter System.
Premature transcription termination at the expanded GAA repeats and aberrant alternative polyadenylation contributes to the Frataxin transcriptional deficit in Friedreich's ataxia.
Salidroside inhibits doxorubicin-induced cardiomyopathy by modulating a ferroptosis-dependent pathway.
Hyperactivation of mTOR and AKT in a cardiac hypertrophy animal model of Friedreich ataxia.
Defective palmitoylation of transferrin receptor triggers iron overload in Friedreich ataxia fibroblasts.
Mitochondrial damage and senescence phenotype of cells derived from a novel frataxin G127V point mutation mouse model of Friedreich's ataxia.
Oxidative stress-dependent frataxin inhibition mediated alcoholic hepatocytotoxicity through ferroptosis.
Quantitative proteomics in Friedreich's ataxia B-lymphocytes: A valuable approach to decipher the biochemical events responsible for pathogenesis.
Autosomal recessive cerebellar ataxia caused by a homozygous mutation in PMPCA.
Intrathecal delivery of frataxin mRNA encapsulated in lipid nanoparticles to dorsal root ganglia as a potential therapeutic for Friedreich's ataxia.
Liu X, Chen L, Lin L, Xie Y, Zheng J, Wang Z, He Z, Wang F, Zeng Q
Biomaterials advances 2026 Aug;185:214842
Biomaterials advances 2026 Aug;185:214842
Antisense oligonucleotide therapy for patients with Friedreich's ataxia carrying the c.165+5G>C splicing mutation.
Yameogo P, Aguilar S, Prakash TP, Rigo F, Lynch DR, Napierala JS, Napierala M
Molecular therapy. Nucleic acids 2025 Sep 9;36(3):102617
Molecular therapy. Nucleic acids 2025 Sep 9;36(3):102617
Analysis and verification of potential ferroptosis-related diagnostic markers in the early stage of metabolic dysfunction-associated steatohepatitis.
Li Q, Zhang Q, Ren A, Zhang H, Liu L
Biochemical and biophysical research communications 2025 Sep 12;779:152406
Biochemical and biophysical research communications 2025 Sep 12;779:152406
Impaired Iron-Sulfur Cluster Synthesis Induces Mitochondrial PARthanatos in Diabetic Cardiomyopathy.
Wang M, Zhang S, Tian J, Yang F, Chen H, Bai S, Kang J, Pang K, Huang J, Dong M, Dong S, Tian Z, Fang S, Fan H, Lu F, Yu B, Li S, Zhang W
Advanced science (Weinheim, Baden-Wurttemberg, Germany) 2025 Jan;12(1):e2406695
Advanced science (Weinheim, Baden-Wurttemberg, Germany) 2025 Jan;12(1):e2406695
Gene editing improves endoplasmic reticulum-mitochondrial contacts and unfolded protein response in Friedreich's ataxia iPSC-derived neurons.
Mishra P, Sivakumar A, Johnson A, Pernaci C, Warden AS, El-Hachem LR, Hansen E, Badell-Grau RA, Khare V, Ramirez G, Gillette S, Solis AB, Guo P, Coufal N, Cherqui S
Frontiers in pharmacology 2024;15:1323491
Frontiers in pharmacology 2024;15:1323491
Reactive oxygen species control protein degradation at the mitochondrial import gate.
McMinimy R, Manford AG, Gee CL, Chandrasekhar S, Mousa GA, Chuang J, Phu L, Shih KY, Rose CM, Kuriyan J, Bingol B, Rapé M
Molecular cell 2024 Dec 5;84(23):4612-4628.e13
Molecular cell 2024 Dec 5;84(23):4612-4628.e13
Design and validation of cell-based potency assays for frataxin supplementation treatments.
Mukherjee S, Pereboeva L, Fil D, Saikia A, Lee J, Li J, Cotticelli MG, Soragni E, Wilson RB, Napierala M, Napierala JS
Molecular therapy. Methods & clinical development 2024 Dec 12;32(4):101347
Molecular therapy. Methods & clinical development 2024 Dec 12;32(4):101347
mTORC1 Signaling Inhibition Modulates Mitochondrial Function in Frataxin Deficiency.
Lehmer M, Zoncu R
bioRxiv : the preprint server for biology 2024 Aug 7;
bioRxiv : the preprint server for biology 2024 Aug 7;
FXN targeting induces cell death in ovarian cancer stem-like cells through PRDX3-Mediated oxidative stress.
Xu S, Liu Y, Yang S, Fei W, Qin J, Lu W, Xu J
iScience 2024 Aug 16;27(8):110506
iScience 2024 Aug 16;27(8):110506
Comparative multi-omic analyses of cardiac mitochondrial stress in three mouse models of frataxin deficiency.
Sayles NM, Napierala JS, Anrather J, Diedhiou N, Li J, Napierala M, Puccio H, Manfredi G
Disease models & mechanisms 2023 Oct 1;16(10)
Disease models & mechanisms 2023 Oct 1;16(10)
Proteomic Study on the Mechanism of Arsenic Neurotoxicity in the Rat Cerebral Cortex and the Protective Mechanism of Dictyophora Polysaccharides against Arsenic Neurotoxicity.
Zhang X, Yang H, Wang Y, Zhang J, Zhang H, Cao X, Hu T, Lin J, Tang X, Yan X, Zhuang H, Chen X, Liang Y, Luo P, Shen L
ACS chemical neuroscience 2023 Jun 21;14(12):2302-2319
ACS chemical neuroscience 2023 Jun 21;14(12):2302-2319
Neurobehavioral deficits of mice expressing a low level of G127V mutant frataxin.
Fil D, Conley RL, Zuberi AR, Lutz CM, Gemelli T, Napierala M, Napierala JS
Neurobiology of disease 2023 Feb;177:105996
Neurobiology of disease 2023 Feb;177:105996
Human frataxin, the Friedreich ataxia deficient protein, interacts with mitochondrial respiratory chain.
Doni D, Cavion F, Bortolus M, Baschiera E, Muccioli S, Tombesi G, d'Ettorre F, Ottaviani D, Marchesan E, Leanza L, Greggio E, Ziviani E, Russo A, Bellin M, Sartori G, Carbonera D, Salviati L, Costantini P
Cell death & disease 2023 Dec 8;14(12):805
Cell death & disease 2023 Dec 8;14(12):805
Iron-frataxin involved in the protective effect of quercetin against alcohol-induced liver mitochondrial dysfunction.
Liu J, Chen H, Lin H, Peng S, Chen L, Cheng X, Yao P, Tang Y
The Journal of nutritional biochemistry 2023 Apr;114:109258
The Journal of nutritional biochemistry 2023 Apr;114:109258
Selected Histone Deacetylase Inhibitors Reverse the Frataxin Transcriptional Defect in a Novel Friedreich's Ataxia Induced Pluripotent Stem Cell-Derived Neuronal Reporter System.
Schreiber AM, Li Y, Chen YH, Napierala JS, Napierala M
Frontiers in neuroscience 2022;16:836476
Frontiers in neuroscience 2022;16:836476
Premature transcription termination at the expanded GAA repeats and aberrant alternative polyadenylation contributes to the Frataxin transcriptional deficit in Friedreich's ataxia.
Li Y, Li J, Wang J, Zhang S, Giles K, Prakash TP, Rigo F, Napierala JS, Napierala M
Human molecular genetics 2022 Oct 10;31(20):3539-3557
Human molecular genetics 2022 Oct 10;31(20):3539-3557
Salidroside inhibits doxorubicin-induced cardiomyopathy by modulating a ferroptosis-dependent pathway.
Chen H, Zhu J, Le Y, Pan J, Liu Y, Liu Z, Wang C, Dou X, Lu D
Phytomedicine : international journal of phytotherapy and phytopharmacology 2022 May;99:153964
Phytomedicine : international journal of phytotherapy and phytopharmacology 2022 May;99:153964
Hyperactivation of mTOR and AKT in a cardiac hypertrophy animal model of Friedreich ataxia.
Tong WH, Ollivierre H, Noguchi A, Ghosh MC, Springer DA, Rouault TA
Heliyon 2022 Aug;8(8):e10371
Heliyon 2022 Aug;8(8):e10371
Defective palmitoylation of transferrin receptor triggers iron overload in Friedreich ataxia fibroblasts.
Petit F, Drecourt A, Dussiot M, Zangarelli C, Hermine O, Munnich A, Rötig A
Blood 2021 Apr 15;137(15):2090-2102
Blood 2021 Apr 15;137(15):2090-2102
Mitochondrial damage and senescence phenotype of cells derived from a novel frataxin G127V point mutation mouse model of Friedreich's ataxia.
Fil D, Chacko BK, Conley R, Ouyang X, Zhang J, Darley-Usmar VM, Zuberi AR, Lutz CM, Napierala M, Napierala JS
Disease models & mechanisms 2020 Jul 27;13(7)
Disease models & mechanisms 2020 Jul 27;13(7)
Oxidative stress-dependent frataxin inhibition mediated alcoholic hepatocytotoxicity through ferroptosis.
Liu J, He H, Wang J, Guo X, Lin H, Chen H, Jiang C, Chen L, Yao P, Tang Y
Toxicology 2020 Dec 1;445:152584
Toxicology 2020 Dec 1;445:152584
Quantitative proteomics in Friedreich's ataxia B-lymphocytes: A valuable approach to decipher the biochemical events responsible for pathogenesis.
Télot L, Rousseau E, Lesuisse E, Garcia C, Morlet B, Léger T, Camadro JM, Serre V
Biochimica et biophysica acta. Molecular basis of disease 2018 Apr;1864(4 Pt A):997-1009
Biochimica et biophysica acta. Molecular basis of disease 2018 Apr;1864(4 Pt A):997-1009
Autosomal recessive cerebellar ataxia caused by a homozygous mutation in PMPCA.
Choquet K, Zurita-Rendón O, La Piana R, Yang S, Dicaire MJ, Care4Rare Consortium, Boycott KM, Majewski J, Shoubridge EA, Brais B, Tétreault M
Brain : a journal of neurology 2016 Mar;139(Pt 3):e19
Brain : a journal of neurology 2016 Mar;139(Pt 3):e19
Intrathecal delivery of frataxin mRNA encapsulated in lipid nanoparticles to dorsal root ganglia as a potential therapeutic for Friedreich's ataxia.
Nabhan JF, Wood KM, Rao VP, Morin J, Bhamidipaty S, LaBranche TP, Gooch RL, Bozal F, Bulawa CE, Guild BC
Scientific reports 2016 Feb 17;6:20019
Scientific reports 2016 Feb 17;6:20019
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Supportive validation
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- The FXN antibody from Proteintech is a rabbit polyclonal antibody to a recombinant protein of human FXN. This antibody recognizes human, mouse, rat antigen. The FXN antibody has been validated for the following applications: ELISA, WB analysis.
