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货号： bs-7759R-RBITC 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-7759R-RBITC
- 英文名称
- Anti-GGCX/RBITC
- 中文名称
- 罗丹明（RBITC）标记的γ-谷氨酰羧化酶抗体
- 别 名
- Gamma glutamyl carboxylase; Gamma-glutamyl carboxylase; GC antibody GGCX; Peptidyl glutamate 4 carboxylase; Peptidyl-glutamate 4-carboxylase; Vitamin K dependent gamma carboxylase; Vitamin K gamma glutamyl carboxylase; Vitamin K-dependent gamma-carboxylase; VKCFD 1; VKCFD1; VKGC_HUMAN.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 心血管 细胞生物
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep,
- 产品应用
- IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 87kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human GGCX
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
This gene encodes an enzyme which catalyzes the posttranslational modification of vitamin K-dependent protein. Many of these vitamin K-dependent proteins are involved in coagulation so the function of the encoded enzyme is essential for hemostasis. Mutations in this gene are associated with vitamin K-dependent coagulation defect and PXE-like disorder with multiple coagulation factor deficiency. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Nov 2008]
Function:
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.
Subunit:
Monomer. May interact with CALU.
Subcellular Location:
Endoplasmic reticulum membrane.
DISEASE:
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.
Similarity:
Belongs to the vitamin K-dependent gamma-carboxylase family.
Database links:Entrez Gene: 2677Human
Omim: 137167Human
SwissProt: P38435Human
Unigene: 77719Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.