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Merck

WH0002395M3

Sigma-Aldrich

Monoclonal Anti-FXN antibody produced in mouse

clone 3G9, purified immunoglobulin, buffered aqueous solution

Synonym(e):

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

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About This Item

MDL-Nummer:
UNSPSC-Code:
12352203
NACRES:
NA.41

Biologische Quelle

mouse

Konjugat

unconjugated

Antikörperform

purified immunoglobulin

Antikörper-Produkttyp

primary antibodies

Klon

3G9, monoclonal

Form

buffered aqueous solution

Speziesreaktivität

human

Methode(n)

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

Isotyp

IgG1κ

GenBank-Hinterlegungsnummer

UniProt-Hinterlegungsnummer

Versandbedingung

dry ice

Lagertemp.

−20°C

Posttranslationale Modifikation Target

unmodified

Angaben zum Gen

human ... FXN(2395)

Allgemeine Beschreibung

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. Wirkung

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.

Physikalische Form

Solution in phosphate buffered saline, pH 7.4

Rechtliche Hinweise

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

Haftungsausschluss

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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Lagerklassenschlüssel

10 - Combustible liquids

Flammpunkt (°F)

Not applicable

Flammpunkt (°C)

Not applicable

Persönliche Schutzausrüstung

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


Analysenzertifikate (COA)

Suchen Sie nach Analysenzertifikate (COA), indem Sie die Lot-/Chargennummer des Produkts eingeben. Lot- und Chargennummern sind auf dem Produktetikett hinter den Wörtern ‘Lot’ oder ‘Batch’ (Lot oder Charge) zu finden.

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Die Dokumentenbibliothek aufrufen

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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