γ-谷氨酰羧化酶抗体
规格：1mg/1ml
英文名： GGCX
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 glu
分子量： 87kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human GGCX
交叉反应：Human, Mouse, Rat,
细胞定位：细胞浆 细胞膜
γ-谷氨酰羧化酶抗体产品介绍：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. γ-谷氨酰羧化酶抗体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, γ-谷氨酰羧化酶抗体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. Gene ID: 2677 Database links: Entrez Gene: 2677 Human Omim: 137167 Human SwissProt: P38435 Human Unigene: 77719 Human 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应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=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.
研究领域：心血管 细胞生物
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid