COG1蛋白抗体
规格：1mg/1ml
英文名： COG1
别名： Ldlbc; CDG2Gv Component of oligomeric golgi complex 1; Conserved oligomeric Golgi complex protein 1; Low density lipoprotein receptor defect B complementing; COG1_HUMAN.
分子量： 109kDa
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 COG1
交叉反应：Human,
细胞定位：细胞浆 细胞膜
COG1蛋白抗体产品介绍：background: There are eight COG proteins (COG1-8) which form a Golgi-localized complex (COG) required for normal Golgi morphology and function. It is thought that COG1 is required for steps in the normal medial and trans Golgi-associated processing of glycoconjugates and plays a role in the organization of the Golgi-localized complex. Function: Required for normal Golgi function (By similarity). Subunit: Component of the conserved oligomeric Golgi complex which is composed of eight different subunits and is required for normal Golgi morphology and localization. Subcellular Location: Golgi apparatus membrane; Peripheral membrane protein; Cytoplasmic side. DISEASE: Defects in COG1 are the cause of congenital disorder of glycosylation type 2G (CDG2G) [MIM:611209]; also known as CDG-II caused by COG1 deficiency.COG1蛋白抗体 CDGs are a family of severe inherited diseases caused by a defect in glycoprotein biosynthesis. They are characterized by under-glycosylated serum glycoproteins. These multisystem disorders present with a wide variety of clinical features, such as disorders of the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions. Clinical features COG1蛋白抗体of CDG2G include failure to thrive, generalized hypotonia, growth retardation and mild psychomotor retardation. CDG2G is biochemically characterized by a defect in O-glycosylation as well as N-glycosylation. Similarity: Belongs to the COG1 family. Database links: UniProtKB/Swiss-Prot: Q8WTW3.1 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
COG1蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 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.
研究领域：细胞生物 信号转导 细胞类型标志物
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid