Recombinant Human QDPR/DHPR protein (ab113148)
Key features and details
- Expression system: Escherichia coli
- Purity: > 90% 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 QDPR/DHPR protein -
精製度
> 90 % SDS-PAGE.
ab113148 was purified using conventional chromatography techniques. -
発現系
Escherichia coli -
アクセッション番号
-
タンパク質長
Full length protein -
Animal free
No -
由来
Recombinant -
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生物種
Human -
予測される分子量
28 kDa including tags -
領域
1 to 244 -
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 ab113148 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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質量分析
MALDI-TOF -
製品の状態
Liquid -
備考
Previously labelled as QDPR.
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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: 0.04% DTT, 0.32% Tris HCl, 10% Glycerol (glycerin, glycerine)
関連情報
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別名
- 6,7 dihydropteridine reductase
- DHPR
- DHPR_HUMAN
see all -
機能
The product of this enzyme, tetrahydrobiopterin (BH-4), is an essential cofactor for phenylalanine, tyrosine, and tryptophan hydroxylases. -
関連疾患
Defects in QDPR are the cause of BH4-deficient hyperphenylalaninemia type C (HPABH4C) [MIM:261630]; also called dihydropteridine reductase deficiency (DHPR deficiency) or hyperphenylalaninemia tetrahydrobiopterin-deficient due to DHPR deficiency or quinoid dihydropteridine reductase deficiency (QDPR deficiency). HPABH4C is a rare autosomal recessive disorder characterized by hyperphenylalaninemia and severe neurologic symptoms (malignant hyperphenylalaninemia) including axial hypotonia and truncal hypertonia, abnormal thermogenesis, and microcephaly. These signs are attributable to depletion of the neurotransmitters dopamine and serotonin, whose syntheses are controlled by tryptophan and tyrosine hydroxylases that use BH-4 as cofactor. These patients do not respond to phenylalanine-restricted diet. HPABH4C is lethal if untreated. -
配列類似性
Belongs to the short-chain dehydrogenases/reductases (SDR) family. - Information by UniProt
画像
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SDS-PAGE - Recombinant Human QDPR/DHPR protein (ab113148)15% SDS-PAGE showing ab113148 (3 µg) at approximately 28.2 kDa.
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)
ab113148 は論文での使用が確認できていません。