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Key Documents

WH0002395M3

Sigma-Aldrich

Monoclonal Anti-FXN antibody produced in mouse

clone 3G9, purified immunoglobulin, buffered aqueous solution

Synonym(s):

Anti-FA, Anti-FARR, Anti-FRDA, Anti-MGC57199, Anti-X25, Anti-frataxin

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About This Item

MDL number:
MFCD02262922
UNSPSC Code:
12352203
NACRES:
NA.41

biological source

mouse

conjugate

unconjugated

antibody form

purified immunoglobulin

antibody product type

primary antibodies

clone

3G9, monoclonal

form

buffered aqueous solution

species reactivity

human

technique(s)

indirect ELISA: suitable
western blot: 1-5 μg/mL

isotype

IgG1κ

GenBank accession no.

NM_000144

UniProt accession no.

Q16595

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... FXN(2395)

General description

Frataxin (FXN) protein consists of α/β fold, which is followed by the C-terminal region (CTR), with a nonperiodic structure.The gene is located on human chromosome 9q21.11.

Immunogen

FXN (AAH48097.1, 91 a.a. ~ 200 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
DETTYERLAEETLDSLAEFFEDLADKPYTFEDYDVSFGSGVLTVKLGGDLGTYVINKQTPNKQIWLSSPSSGPKRYDWTGKNWVYSHDGVSLHELLAAELTKALKTKLDL

Biochem/physiol Actions

Frataxin (FXN) is involved in the activation of iron-sulfur cluster assembly.Overexpression of FXN in reticulocytes is associated with oxidative stress and iron status.Deficiency of FXN in human astrocytes is linked to cell death and release of neurotoxins.

Physical form

Solution in phosphate buffered saline, pH 7.4

Legal Information

GenBank is a registered trademark of United States Department of Health and Human Services

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Storage Class Code

10 - Combustible liquids

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Personal Protective Equipment

dust mask type N95 (US), Eyeshields, Gloves

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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Evidence for chromosome fragility at the frataxin locus in Friedreich ataxia
Kumari,, et al.
Mutation Research. Fundamental and Molecular Mechanisms of Mutagenesis, 781, 14-21 (2015)
The alteration of the C-terminal region of human frataxin distorts its structural dynamics and function
Faraj, S, et al.
FEBS Journal, 281(15), 3397-3419 (2014)
Frataxin expression in reticulocytes of non-splenectomized and splenectomized patients with HbE-beta-thalassaemia
Suebpeng, et al.
Clinical Biochemistry, 49(6), 463-466 (2016)
Insights on the conformational dynamics of human frataxin through modifications of loop-1.
Noguera ME, et al.
Archives of Biochemistry and Biophysics, 636, 123-137 (2017)
Frida Loría et al.
Neurobiology of disease, 76, 1-12 (2015-01-03)
Friedreich's ataxia (FA) is a recessive, predominantly neurodegenerative disorder caused in most cases by mutations in the first intron of the frataxin (FXN) gene. This mutation drives the expansion of a homozygous GAA repeat that results in decreased levels of

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