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Principaux documents

COO/COA

WH0002395M3

Monoclonal Anti-FXN antibody produced in mouse

Name: Monoclonal Anti-FXN antibody produced in mouse
Brand: SIGMA

clone 3G9, purified immunoglobulin, buffered aqueous solution

Synonyme(s) :

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

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About This Item

Numéro MDL:

MFCD02262922

Code UNSPSC :

12352203

Nomenclature NACRES :

NA.41

Source biologique

mouse

Niveau de qualité

100

Conjugué

unconjugated

Forme d'anticorps

purified immunoglobulin

Type de produit anticorps

primary antibodies

Clone

3G9, monoclonal

Forme

buffered aqueous solution

Espèces réactives

human

Technique(s)

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

Isotype

IgG1κ

Numéro d'accès GenBank

NM_000144

Numéro d'accès UniProt

Q16595

Conditions d'expédition

dry ice

Température de stockage

−20°C

Modification post-traductionnelle de la cible

unmodified

Informations sur le gène

human ... FXN(2395)

Description générale

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.

Immunogène

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

Actions biochimiques/physiologiques

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.

Forme physique

Solution in phosphate buffered saline, pH 7.4

Informations légales

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

Clause de non-responsabilité

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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Code de la classe de stockage

10 - Combustible liquids

Point d'éclair (°F)

Not applicable

Point d'éclair (°C)

Not applicable

Équipement de protection individuelle

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)

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Certificats d'analyse (COA)

Lot/Batch Number

07138-3G9

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Consulter la Bibliothèque de documents

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)

Frataxin expression in reticulocytes of non-splenectomized and splenectomized patients with HbE-beta-thalassaemia

Suebpeng, et al.

Clinical Biochemistry, 49(6), 463-466 (2016)

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)

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)

Frataxin knockdown in human astrocytes triggers cell death and the release of factors that cause neuronal toxicity.

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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