Native人Fibrinogen蛋白(ab62253)
Key features and details
- Expression system: Native
- Purity: > 95% Ion Exchange Chromatography
描述
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产品名称
Native人Fibrinogen蛋白
参阅全部 Fibrinogen 蛋白酶 -
纯度
> 95 % Ion Exchange Chromatography.
Isolated by salt fractionation, gel filtration and ion exchange chromatography. -
表达系统
Native -
Accession
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蛋白长度
Protein fragment -
无动物成分
No -
性质
Native -
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氨基酸序列 1
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种属
Human
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氨基酸序列 2
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种属
Human
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氨基酸序列 3
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种属
Human
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技术指标
Our Abpromise guarantee covers the use of ab62253 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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形式
Lyophilized -
Concentration information loading...
制备和贮存
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稳定性和存储
Shipped at 4°C. Upon delivery aliquot. Store at +4°C. Avoid freeze / thaw cycle.
Constituents: 0.9% Sodium chloride, 3% Glycine
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复溶Add 1 ml of water for a final concentration of 0.1 mg/ml.
常规信息
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别名
- FGA
- FGB
- FGG
see all -
功能
Fibrinogen has a double function: yielding monomers that polymerize into fibrin and acting as a cofactor in platelet aggregation. -
组织特异性
Plasma. -
疾病相关
Defects in FGA are a cause of congenital afibrinogenemia (CAFBN) [MIM:202400]. This is a rare autosomal recessive disorder characterized by bleeding that varies from mild to severe and by complete absence or extremely low levels of plasma and platelet fibrinogen. Note=The majority of cases of afibrinogenemia are due to truncating mutations. Variations in position Arg-35 (the site of cleavage of fibrinopeptide a by thrombin) leads to alpha-dysfibrinogenemias.
Defects in FGA are a cause of amyloidosis type 8 (AMYL8) [MIM:105200]; also known as systemic non-neuropathic amyloidosis or Ostertag-type amyloidosis. AMYL8 is a hereditary generalized amyloidosis due to deposition of apolipoprotein A1, fibrinogen and lysozyme amyloids. Viscera are particularly affected. There is no involvement of the nervous system. Clinical features include renal amyloidosis resulting in nephrotic syndrome, arterial hypertension, hepatosplenomegaly, cholestasis, petechial skin rash. -
序列相似性
Contains 1 fibrinogen C-terminal domain. -
结构域
A long coiled coil structure formed by 3 polypeptide chains connects the central nodule to the C-terminal domains (distal nodules). The long C-terminal ends of the alpha chains fold back, contributing a fourth strand to the coiled coil structure. -
翻译后修饰
The alpha chain is not glycosylated.
Forms F13A-mediated cross-links between a glutamine and the epsilon-amino group of a lysine residue, forming fibronectin-fibrinogen heteropolymers.
About one-third of the alpha chains in the molecules in blood were found to be phosphorylated.
Conversion of fibrinogen to fibrin is triggered by thrombin, which cleaves fibrinopeptides A and B from alpha and beta chains, and thus exposes the N-terminal polymerization sites responsible for the formation of the soft clot. The soft clot is converted into the hard clot by factor XIIIA which catalyzes the epsilon-(gamma-glutamyl)lysine cross-linking between gamma chains (stronger) and between alpha chains (weaker) of different monomers.
Phosphorylation sites are present in the extracellular medium. -
细胞定位
Secreted. - Information by UniProt
实验方案
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
数据表及文件
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Datasheet download
文献 (0)
ab62253 尚未被引用在任何文献中。