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Frataxin Polyclonal antibody

Frataxin Polyclonal Antibody for WB, IHC, ELISA

Host / Isotype

Rabbit / IgG

Reactivity

human, mouse, rat

Applications

WB, IHC, ELISA and More (2)

Conjugate

Unconjugated

Cat no : 14147-1-AP

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Synonyms

FXN, FA, Extramitochondrial frataxin, EC:1.16.3.1, dFXN



经过测试的应用

Positive WB detected inHCT 116 cells, Jurkat cells, K-562 cells, Raji cells, mouse liver tissue
Positive IHC detected inhuman stomach tissue
Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0

推荐稀释比

ApplicationDilution
Western Blot (WB)WB : 1:1000-1:6000
Immunohistochemistry (IHC)IHC : 1:300-1:1200
It is recommended that this reagent should be titrated in each testing system to obtain optimal results.
Sample-dependent, Check data in validation data gallery.

产品信息

14147-1-AP targets Frataxin in WB, IHC, IF, CoIP, ELISA applications and shows reactivity with human, mouse, rat samples.

Tested Applications WB, IHC, ELISA Application Description
Cited ApplicationsWB, IHC, IF, CoIP
Tested Reactivity human, mouse, rat
Cited Reactivityhuman, mouse, rat
Immunogen Frataxin fusion protein Ag5316 种属同源性预测
Host / Isotype Rabbit / IgG
Class Polyclonal
Type Antibody
Full Name frataxin
Synonyms FXN, FA, Extramitochondrial frataxin, EC:1.16.3.1, dFXN
Calculated Molecular Weight 23 kDa
Observed Molecular Weight 14 kDa
GenBank Accession NumberBC048097
Gene Symbol FXN
Gene ID (NCBI) 2395
RRIDAB_2231876
Conjugate Unconjugated
Form Liquid
Purification MethodAntigen affinity purification
UNIPROT IDQ16595
Storage Buffer PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
Storage ConditionsStore at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage.

背景介绍

Frataxin (FXN), also named as FRDA, X25, m81-FXN, d-FXN, m78-FXN and i-FXN, belongs to the frataxin family. It promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe2+ to proteins involved in these pathways. FXN may play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe2+ to Fe3+; the oligomeric form but not the monomeric form has in vitro ferroxidase activity. FXN is cleaved to be 4 chains. FXN is expressed in the cytoplasm as a 210 amino acid (AA) precursor protein (pFXN; 23 KDa) that is translocated into mitochondria where it is processed by two consecutive steps into iFXN (FXN 42-210; 19 KDa) and finally mFXN (81-210; 14.2 KDa), which is functional. (PMID: 26704351, PMID: 26671574)

实验方案

Product Specific Protocols
WB protocol for Frataxin antibody 14147-1-APDownload protocol
IHC protocol for Frataxin antibody 14147-1-APDownload protocol
Standard Protocols
Click here to view our Standard Protocols

发表文章

SpeciesApplicationTitle
humanWB

Blood

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

Authors - Floriane Petit
humanWB

Brain

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

Authors - Karine Choquet
mouseWB

Neurobiol Dis

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

Authors - Daniel Fil
mouseWB

J Nutr Biochem

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

Authors - Jingjing Liu
humanWB,IHC

Sci Rep

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

Authors - Joseph F Nabhan
humanWB

Hum Mol Genet

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

Authors - Yanjie Li