Proteintech

FXN Polyclonal Antibody

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FXN Antibody (14147-1-AP) in WB

Human cerebellum tissue were subjected to SDS PAGE followed by western blot with 14147-1-AP (FXN antibody) at dilution of 1:500 incubated at room temperature for 1.5 hours.

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Product Details

14147-1-AP

Applications

Tested Dilution

Publications

Western Blot (WB)

1:500-1:2,000

View 11 publications 11 publications

Immunohistochemistry (IHC)

Assay-dependent

View 1 publication 1 publication

Miscellaneous PubMed (MISC)

View 1 publication 1 publication

Not applicable (N/A)

View 1 publication 1 publication

Product Specifications

Species Reactivity

Human, Mouse, Rat

Published species

Human

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

FXN Fusion Protein Ag5316 (1-210 aa encoded by BC048097)

View immunogen

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

0.13 mg/mL

Purification

Antigen affinity chromatography

Storage buffer

PBS, pH 7.3, with 50% glycerol

Contains

0.02% sodium azide

Storage conditions

-20°C

Shipping conditions

Wet ice (domestic); Dry ice (international)

Product Specific Information

Immunogen sequence: MWTLGRRAV AGLLASPSPA QAQTLTRVPR PAELAPLCGR RGLRTDIDAT CTPRRASSNQ RGLNQIWNVK KQSVYLMNLR KSGTLGHPGS LDETTYERLA EETLDSLAEF FEDLADKPYT FEDYDVSFGS GVLTVKLGGD LGTYVINKQT PNKQIWLSSP SSGPKRYDWT GKNWVYSHDG VSLHELLAAE LTKALKTKLD LSSLAYSGKD A (1-210 aa encoded by BC048097)

Target Information

FXN is 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 results in Friedreich ataxia.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.

Product References

Iron-frataxin involved in the protective effect of quercetin against alcohol-induced liver mitochondrial dysfunction.

The Journal of nutritional biochemistry

Liu J,Chen H,Lin H,Peng S,Chen L,Cheng X,Yao P,Tang Y

Sat Apr 01 00:00:00 UTC 2023

Neurobehavioral deficits of mice expressing a low level of G127V mutant frataxin.

Neurobiology of disease

Fil D,Conley RL,Zuberi AR,Lutz CM,Gemelli T,Napierala M,Napierala JS

Wed Feb 01 00:00:00 UTC 2023

Salidroside inhibits doxorubicin-induced cardiomyopathy by modulating a ferroptosis-dependent pathway.

Phytomedicine : international journal of phytotherapy and phytopharmacology

Chen H,Zhu J,Le Y,Pan J,Liu Y,Liu Z,Wang C,Dou X,Lu D

Sun May 01 00:00:00 UTC 2022

Selected Histone Deacetylase Inhibitors Reverse the Frataxin Transcriptional Defect in a Novel Friedreich's Ataxia Induced Pluripotent Stem Cell-Derived Neuronal Reporter System.

Frontiers in neuroscience

Schreiber AM,Li Y,Chen YH,Napierala JS,Napierala M

Wed Jan 24 00:00:00 UTC 2024

Premature transcription termination at the expanded GAA repeats and aberrant alternative polyadenylation contributes to the Frataxin transcriptional deficit in Friedreich's ataxia.

Human molecular genetics

Li Y,Li J,Wang J,Zhang S,Giles K,Prakash TP,Rigo F,Napierala JS,Napierala M

Mon Oct 10 00:00:00 UTC 2022

Hyperactivation of mTOR and AKT in a cardiac hypertrophy animal model of Friedreich ataxia.

Heliyon

Tong WH,Ollivierre H,Noguchi A,Ghosh MC,Springer DA,Rouault TA

Mon Aug 01 00:00:00 UTC 2022

Defective palmitoylation of transferrin receptor triggers iron overload in Friedreich ataxia fibroblasts.

Blood

Petit F,Drecourt A,Dussiot M,Zangarelli C,Hermine O,Munnich A,Rötig A

Thu Apr 15 00:00:00 UTC 2021

Oxidative stress-dependent frataxin inhibition mediated alcoholic hepatocytotoxicity through ferroptosis.

Toxicology

Liu J,He H,Wang J,Guo X,Lin H,Chen H,Jiang C,Chen L,Yao P,Tang Y

Tue Dec 01 00:00:00 UTC 2020

Mitochondrial damage and senescence phenotype of cells derived from a novel frataxin G127V point mutation mouse model of Friedreich's ataxia.

Disease models & mechanisms

Fil D,Chacko BK,Conley R,Ouyang X,Zhang J,Darley-Usmar VM,Zuberi AR,Lutz CM,Napierala M,Napierala JS

Mon Jul 27 00:00:00 UTC 2020

Quantitative proteomics in Friedreich's ataxia B-lymphocytes: A valuable approach to decipher the biochemical events responsible for pathogenesis.

Biochimica et biophysica acta. Molecular basis of disease

Télot L,Rousseau E,Lesuisse E,Garcia C,Morlet B,Léger T,Camadro JM,Serre V

Sun Apr 01 00:00:00 UTC 2018

Quantitative proteomics in Friedreich's ataxia B-lymphocytes: A valuable approach to decipher the biochemical events responsible for pathogenesis.

Biochimica et biophysica acta. Molecular basis of disease

Télot L,Rousseau E,Lesuisse E,Garcia C,Morlet B,Léger T,Camadro JM,Serre V

Sun Apr 01 00:00:00 UTC 2018

Autosomal recessive cerebellar ataxia caused by a homozygous mutation in PMPCA.

Brain : a journal of neurology

Choquet K,Zurita-Rendón O,La Piana R,Yang S,Dicaire MJ,Boycott KM,Majewski J,Shoubridge EA,Brais B,Tétreault M

Tue Mar 01 00:00:00 UTC 2016

Autosomal recessive cerebellar ataxia caused by a homozygous mutation in PMPCA.

Brain : a journal of neurology

Choquet K,Zurita-Rendón O,La Piana R,Yang S,Dicaire MJ,Boycott KM,Majewski J,Shoubridge EA,Brais B,Tétreault M

Tue Mar 01 00:00:00 UTC 2016

Intrathecal delivery of frataxin mRNA encapsulated in lipid nanoparticles to dorsal root ganglia as a potential therapeutic for Friedreich's ataxia.

Scientific reports

Nabhan JF,Wood KM,Rao VP,Morin J,Bhamidipaty S,LaBranche TP,Gooch RL,Bozal F,Bulawa CE,Guild BC

Wed Feb 17 00:00:00 UTC 2016

Intrathecal delivery of frataxin mRNA encapsulated in lipid nanoparticles to dorsal root ganglia as a potential therapeutic for Friedreich's ataxia.

Scientific reports

Nabhan JF,Wood KM,Rao VP,Morin J,Bhamidipaty S,LaBranche TP,Gooch RL,Bozal F,Bulawa CE,Guild BC

Wed Feb 17 00:00:00 UTC 2016

Intrathecal delivery of frataxin mRNA encapsulated in lipid nanoparticles to dorsal root ganglia as a potential therapeutic for Friedreich's ataxia.

Scientific reports

Nabhan JF,Wood KM,Rao VP,Morin J,Bhamidipaty S,LaBranche TP,Gooch RL,Bozal F,Bulawa CE,Guild BC

Wed Feb 17 00:00:00 UTC 2016

Intrathecal delivery of frataxin mRNA encapsulated in lipid nanoparticles to dorsal root ganglia as a potential therapeutic for Friedreich's ataxia.

Scientific reports

Nabhan JF,Wood KM,Rao VP,Morin J,Bhamidipaty S,LaBranche TP,Gooch RL,Bozal F,Bulawa CE,Guild BC

Wed Feb 17 00:00:00 UTC 2016

Bioinformatics

Protein Aliases: Frataxin, mitochondrial; Friedreich ataxia; Friedreich ataxia protein; Fxn

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Gene Aliases: CyaY; FA; FARR; FRDA; FXN; RGD1565754; X25

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UniProt ID: (Human) Q16595, (Mouse) O35943

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Entrez Gene ID: (Human) 2395, (Rat) 499335, (Mouse) 14297

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Function(s)

ferroxidase activity protein binding ferrous iron binding ferric iron binding iron chaperone activity iron-sulfur cluster binding 2 iron, 2 sulfur cluster binding metallochaperone activity enzyme binding oxidoreductase activity metal ion binding

Process(es)

Performance Guarantee

If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*

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FXN Polyclonal Antibody

FXN Antibody (14147-1-AP) in WB

Product Details

14147-1-AP

Western Blot (WB)

Immunohistochemistry (IHC)

Miscellaneous PubMed (MISC)

Not applicable (N/A)

Species Reactivity

Published species

Host/Isotype

Class

Type

Immunogen

Conjugate

Form

Concentration

Purification

Storage buffer

Contains

Storage conditions

Shipping conditions

Product Specific Information

Target Information

Bioinformatics

Performance Guarantee

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