产品名称
FXN Rabbit Polyclonal Antibody
蛋白名称
Frataxin, mitochondrial (EC 1.16.3.1) (Friedreich ataxia protein) (Fxn) [Cleaved into: Frataxin intermediate form (i-FXN); Frataxin(56-210) (m56-FXN); Frataxin(78-210) (d-FXN) (m78-FXN); Frataxin mature form (Frataxin(81-210)) (m81-FXN)]
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=2395
Human Swissprot No.
Q16595
Human Swissprot Link
https://www.uniprot.org/uniprot/Q16595
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=14297
Mouse Swissprot No.
O35943
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/O35943/entry
Rat Swissprot Link
https://www.uniprot.org/uniprotkb/D3ZYW7/entry
特异性
This antibody detects endogenous levels of FXN at Human, Mouse,Rat
运输及保存条件
-15°C to -25°C/1 year(Do not lower than -25°C)
组织表达
Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
细胞定位
Mitochondrion . Cytoplasm, cytosol . PubMed:18725397 reports localization exclusively in mitochondria. .
功能
Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization; however, the physiological relevance is unsure as reports are conflicting and the function has only been shown using heterologous overexpression systems. Modulates the RNA-binding activity of ACO1.
