Recombinant Human Frataxin protein (ab95502)
Key features and details
- Expression system: Escherichia coli
- Purity: > 95% SDS-PAGE
- Tags: His tag N-Terminus
- Suitable for: SDS-PAGE, MS
製品の詳細
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製品名
Recombinant Human Frataxin protein
Frataxin タンパク質・ペプチド 製品一覧 -
精製度
> 95 % SDS-PAGE.
ab95502 is purified using conventional chromatography techniques. -
発現系
Escherichia coli -
アクセッション番号
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タンパク質長
Full length protein -
Animal free
No -
由来
Recombinant -
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生物種
Human -
配列
MGSSHHHHHH SSGLVPRGSH MLRTDIDATC TPRRASSNQR GLNQIWNVKK QSVYLMNLRK SGTLGHPGSL DETTYERLAE ETLDSLAEFF EDLADKPYTF EDYDVSFGSG VLTVKLGGDL GTYVINKQTP NKQIWLSSPS SGPKRYDWTG KNWVYSHDGV SLHELLAAEL TKALKTKLDL SSLAYSGKDA -
予測される分子量
21 kDa including tags -
実際の分子量
21 kDa including tags -
領域
42 to 210 -
タグ
His tag N-Terminus
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関連製品
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Related Products
特性
Our Abpromise guarantee covers the use of ab95502 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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アプリケーション
SDS-PAGE
Mass Spectrometry
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製品の状態
Liquid -
Concentration information loading...
前処理および保存
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保存方法および安定性
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
pH: 8.5
Constituents: 0.0154% DTT, 0.316% Tris HCl, 10% Glycerol (glycerin, glycerine)
関連情報
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別名
- CyaY
- d-FXN
- FA
see all -
機能
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. -
組織特異性
Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts. -
関連疾患
Defects in FXN are the cause of Friedreich ataxia (FRDA) [MIM:229300]. FRDA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually manifest before adolescence and is generally characterized by incoordination of limb movements, dysarthria, nystagmus, diminished or absent tendon reflexes, Babinski sign, impairment of position and vibratory senses, scoliosis, pes cavus, and hammer toe. In most patients, FRDA is due to GAA triplet repeat expansions in the first intron of the frataxin gene. But in some cases the disease is due to mutations in the coding region. -
配列類似性
Belongs to the frataxin family. -
翻訳後修飾
Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to yield frataxin mature form (frataxin(81-210)) which is the predominant form. The additional forms, frataxin(56-210) and frataxin(78-210), seem to be produced when the normal maturation process is impaired; their physiological relevance is unsure. -
細胞内局在
Cytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria. - Information by UniProt
プロトコール
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
データシートおよび資料
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SDS download
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Datasheet download
参考文献 (1)
ab95502 は 1 報の論文で使用されています。
- Anderson BM et al. N-Terminomics/TAILS Profiling of Macrophages after Chemical Inhibition of Legumain. Biochemistry 59:329-340 (2020). PubMed: 31774660