一般描述
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.
免疫原
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
Sequence
DETTYERLAEETLDSLAEFFEDLADKPYTFEDYDVSFGSGVLTVKLGGDLGTYVINKQTPNKQIWLSSPSSGPKRYDWTGKNWVYSHDGVSLHELLAAELTKALKTKLDL
生化/生理作用
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.
外形
Solution in phosphate buffered saline, pH 7.4
法律信息
GenBank is a registered trademark of United States Department of Health and Human Services
免责声明
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.
闪点(°F)
Not applicable
闪点(°C)
Not applicable
个人防护装备
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
法规信息
新产品
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)
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)
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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