Antibody data
- Antibody Data
- Antigen structure
- References [16]
- Comments [0]
- Validations
- Western blot [1]
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Validation data
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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 Gene editing improves endoplasmic reticulum-mitochondrial contacts and unfolded protein response in Friedreich's ataxia iPSC-derived neurons.
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.
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
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.