FITC标记的COG1蛋白抗体上海沪震实业有限公司

性能参数

产品名称: FITC标记的COG1蛋白抗体
英文名称: Anti-COG1/FITC
抗体货号: HZ-6647R-FITC
产品规格: 100ul
级    别: 分析纯, , 分析纯,
产品产地: 中国/上海
品牌商标: HZbscience
价    格: 2980元
抗原: Rabbit
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: 
FITCconjugate:FITC
应用范围: IF=1:50-200
更新时间: 2018/7/13 9:28:00
详细资料:  实验方法技术资料
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诚信指数:788点
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使用范围:仅限科研使用,不能应用于临床
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供应商联系卡

上海沪震实业有限公司
地址:
上海市杨浦区密云路1018号复旦科技园808室
邮编:
200612
电话:
021-60345367
传真:
021-31320307
联系人:
陈先生
所在区域:
上海·中国-上海
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产品详细描述

 Rabbit Anti-COG1/FITC Conjugated antibody 

FITC标记的COG1蛋白抗体

 

英文名称 Anti-COG1/FITC
中文名称 FITC标记的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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
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, Rat, 
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 109kDa
细胞定位 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
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.
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.

Database links:

Entrez Gene: 9382 Human

Entrez Gene: 16834 Mouse

Omim: 606973 Human

SwissProt: Q8WTW3 Human

SwissProt: Q9Z160 Mouse

Unigene: 103555 Human

Unigene: 261620 Mouse



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

有八个COG蛋白(COG1-8),形成高尔基局部复合物(COG)所需的正常高尔基形态和功能。据认为,COG1是需要在正常的内侧和跨高尔基体相关的糖复合物处理的步骤,并在高尔基体局部复合物的组织中起作用。

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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