COG1,COG1蛋白抗体
产品名称: COG1,COG1蛋白抗体
英文名称: Anti-COG1 antibody
产品编号: HZ-6647R
产品价格: null
产品产地: 中国/美国
品牌商标: HZbscience
更新时间: 2023-08-17T10:24:20
使用范围: WB,ELISA,IHC-P,IHC-F,IF
- 联系人 : 鲍丽雯
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COG1,COG1蛋白抗体
产品编号HZ-6647R
英文名称COG1
中文名称COG1蛋白抗体
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.
说 明 书0.1ml 0.2ml
研究领域细胞生物 信号转导 细胞类型标志物
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human,
COG1,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.
分 子 量109kDa
细胞定位细胞浆 细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human COG1
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.
COG1,COG1蛋白抗体PubMedPubMed
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. 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 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.
COG1,COG1蛋白抗体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.