γ-谷氨酰羧化酶抗体

γ-谷氨酰羧化酶抗体英文名称  Anti-GGCX 

中文名称  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. 

浓    度  1mg/1ml 

规 格  0.2ml/200μg     

纯化的抗体可通过不同的途径获取，有些γ-谷氨酰羧化酶抗体可通过下述方法制备或从商家购买。从商家购买的抗体，通常附有正确的储存方法。

1）工作液应在4℃下融化并存放，可能稳定达数月。

2）如果没有特殊原因而避免使用叠氮钠，亦可加入叠氮钠，浓度为0．02％。将纯化的抗体样本分装成合适的体积，于-20℃保存。

3）纯化的抗体溶液应以较高的浓度(如lmg／m1)在中性pH下保存。：常用的抗体储存浓度高达l0mg/ml。较低浓度的抗体冻存前应浓缩。所有标准的浓缩方法(如超滤法)，皆可使用。还有一个简单的方法是用蛋白A或蛋白G亲和柱来浓缩溶液。如果纯化的抗体不是用于标记，可将它们以较低浓度储存于加有1％BSA的溶液中。

4）经纯化制备的抗体在常用的缓冲液中是稳定的。其DH应保持在中性左右。如果pH在7-8之间，即使保存多年，对抗体也无损害。多数情况下，盐浓度适于保持在0-150mmol/L之间，但在长期存放的抗体中，盐溶液浓度高达500mmol/L时，对γ-谷氨酰羧化酶抗体能有损害。如果没有其他说明．律议用PBS或50mmol/LTris(DH8．0)溶液长期存放抗体。  

抗体来源  Rabbit  

克隆类型  polyclonal 

交叉反应  Human, Mouse, Rat, Horse

产品类型  一抗    

研究领域  心血管 细胞生物  

蛋白分子量  predicted molecular weight: 87kDa 

性    状  Lyophilized or Liquid 

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  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:100-500 

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

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. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

γ-谷氨酰羧化酶抗体产品介绍 GGCX is a 758 amino acid multi-pass membrane protein. Localized to the membrane of the endoplasmic reticulum, GGCX functions to mediate the vitamin K-dependent carboxylation of glutamate residues on target proteins, thereby producing calcium binding gamma-carboxyglutamate (Gla) residues on these proteins and simultaneously converting vitamin K to vitamin K epoxide. GGCX exists as a monomer and, via its ability to modify glutamate residues, it accomplishes the post-translational changes that are necessary for the activity of all vitamin K-dependent proteins (such as blood coagulation and bone matrix proteins). Defects in the gene encoding GGCX are the cause of combined deficiency of vitamin K-dependent clotting factors 1 (VKCFD1) and PXE-like disorder with multiple coagulation factor deficiency, both of which are characterized by abnormal skin, blood or bone function. 

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 (By similarity).

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 : UniProtKB/Swiss-Prot: P38435.2