Recombinant Human GGCX protein (ab114658)
Key features and details
- Expression system: Wheat germ
- Suitable for: WB, SDS-PAGE, ELISA
製品の詳細
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製品名
Recombinant Human GGCX protein -
発現系
Wheat germ -
アクセッション番号
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タンパク質長
Protein fragment -
Animal free
No -
由来
Recombinant -
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生物種
Human -
配列
ADFPGLHLENFVSEDLGNTSIQLLQGEVTVELVAEQKNQTLREGEKMQLP AGEYHKVYTTSPSPSCYMYVYVNTTELALEQDLAYLQELKEKVENGS -
予測される分子量
36 kDa including tags -
領域
533 to 629
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特性
Our Abpromise guarantee covers the use of ab114658 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
Western blot
SDS-PAGE
ELISA
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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 on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00
Constituents: 0.3% Glutathione, 0.79% Tris HCl
関連情報
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別名
- FLJ26629
- Gamma glutamyl carboxylase
- Gamma-glutamyl carboxylase
see all -
機能
Mediates the vitamin K-dependent carboxylation of glutamate residues to calcium-binding gamma-carboxyglutamate (Gla) residues with the concomitant conversion of the reduced hydroquinone form of vitamin K to vitamin K epoxide. -
関連疾患
Defects in GGCX are a cause of combined deficiency of vitamin K-dependent clotting factors type 1 (VKCFD1) [MIM:277450]; also known as multiple coagulation factor deficiency III (MCFD3). VKCFD leads to a bleeding tendency that is usually reversed by oral administration of vitamin K.
Defects in GGCX are the cause of pseudoxanthoma elasticum-like disorder with multiple coagulation factor deficiency (PXEL-MCFD) [MIM:610842]. This syndrome is characterized by hyperlaxity of the skin involving the entire body. Important phenotypic differences with classical PXE include much more severe skin laxity with spreading toward the trunk and limbs with thick, leathery skin folds rather than confinement to flexural areas, and no decrease in visual acuity. Moreover, detailed electron microscopic analyzes revealed that alterations of elastic fibers as well as their mineralization are slightly different from those in classic PXE. -
配列類似性
Belongs to the vitamin K-dependent gamma-carboxylase family. -
細胞内局在
Endoplasmic reticulum membrane. - 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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Datasheet download
参考文献 (0)
ab114658 は論文での使用が確認できていません。