Rabbit Anti-COG1/FITC Conjugated antibody
|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|
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
|分 子 量||109kDa|
|性 状||Lyophilized or Liquid|
|免 疫 原||KLH conjugated synthetic peptide derived from human COG1|
|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.|
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.
Required for normal Golgi function (By similarity).
Component of the conserved oligomeric Golgi complex which is composed of eight different subunits and is required for normal Golgi morphology and localization.
Golgi apparatus membrane; Peripheral membrane protein; Cytoplasmic side.
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.
Belongs to the COG1 family.
Entrez Gene: 9382 Human
Entrez Gene: 16834 Mouse
Omim: 606973 Human
SwissProt: Q8WTW3 Human
SwissProt: Q9Z160 Mouse
Unigene: 103555 Human
Unigene: 261620 Mouse
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications