Recombinant Human NFU1 protein (ab187452)
Key features and details
- Expression system: Escherichia coli
- Purity: > 90% SDS-PAGE
- Tags: His tag N-Terminus
- Suitable for: MS, SDS-PAGE
製品の詳細
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製品名
Recombinant Human NFU1 protein -
精製度
> 90 % SDS-PAGE. -
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
MGSSHHHHHHSSGLVPRGSHMGSGAAAVAAGLRRRFCHMLKNPYTIKKQP LHQFVQRPLFPLPAAFYHPVRYMFIQTQDTPNPNSLKFIPGKPVLETRTM DFPTPAAAFRSPLARQLFRIEGVKSVFFGPDFITVTKENEELDWNLLKPD IYATIMDFFASGLPLVTEETPSGEAGSEEDDEVVAMIKELLDTRIRPTVQ EDGGDVIYKGFEDGIVQLKLQGSCTSCPSSIITLKNGIQNMLQFYIPEVE GVEQVMDDESDEKEANSP -
予測される分子量
30 kDa including tags -
領域
10 to 254 -
タグ
His tag N-Terminus -
配列の追加情報
Full length mature protein
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関連製品
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Related Products
特性
Our Abpromise guarantee covers the use of ab187452 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
Mass Spectrometry
SDS-PAGE
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質量分析
MALDI-TOF -
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. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
pH: 7.40
Constituents: PBS, 20% Glycerol (glycerin, glycerine)
関連情報
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別名
- 0610006G17Rik
- CGI 33
- HIRA interacting protein 5
see all -
機能
Iron-sulfur cluster scaffold protein which can assemble [4Fe-2S] clusters and deliver them to target proteins. -
組織特異性
Ubiquitous. Expression in adult lung is weak compared to fetal lung. -
関連疾患
Defects in NFU1 are the cause of multiple mitochondrial dysfunctions syndrome type 1 (MMDS1) [MIM:605711]. A severe disorder of systemic energy metabolism, resulting in weakness, respiratory failure, lack of neurologic development, lactic acidosis, hyperglycinemia and early death. Some patients show failure to thrive, pulmonary hypertension, hypotonia and irritability. Biochemical features include severe combined deficiency of the 2-oxoacid dehydrogenases, defective lipoic acid synthesis and reduction in activity of mitochondrial respiratory chain complexes. -
配列類似性
Belongs to the NifU family. -
発生段階
Expressed in embryo and adult. -
細胞内局在
Mitochondrion. Cytoplasm > cytosol. - Information by UniProt
画像
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SDS-PAGE - Recombinant Human NFU1 protein (ab187452)
15% SDS-PAGE analysis of 3µg ab187452.
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 (0)
ab187452 は論文での使用が確認できていません。