Fibrinogen gamma chain,纤维蛋白原γ链抗体

产品编号HZ-6895R
英文名称Fibrinogen gamma chain
中文名称纤维蛋白原γ链抗体
别 名FGG; FIBG_HUMAN; Fibrinogen gamma chain; Fibrinogen gamma polypeptide; fibrinogen gamma-b chain.
说 明 书0.1ml 0.2ml
研究领域心血管 细胞生物 免疫学
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Horse, Rabbit,
Fibrinogen gamma chain,纤维蛋白原γ链抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=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47kDa
细胞定位分泌型蛋白
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human Fibrinogen gamma chain
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.
Fibrinogen gamma chain,纤维蛋白原γ链抗体PubMedPubMed
产品介绍background:
Fibrinogen has a double function: yielding monomers that polymerize into fibrin and acting as a cofactor in platelet aggregation.
Involvement in disease: Defects in FGG are a cause of thrombophilia. Defects in FGG are a cause of congenital afibrinogenemia (CAFBN). It is a rare autosomal recessive disorder characterized by complete absence of detectable fibrinogen.

Function:
Fibrinogen has a double function: yielding monomers that polymerize into fibrin and acting as a cofactor in platelet aggregation.

Subunit:
Heterohexamer; disulfide linked. Contains 2 sets of 3 non-identical chains (alpha, beta and gamma). The 2 heterotrimers are in head to head conformation with the N-termini in a small central domain.

Subcellular Location:
Secreted.

Fibrinogen gamma chain,纤维蛋白原γ链抗体Post-translational modifications:
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.
Sulfation of C-terminal tyrosines increases affinity for thrombin.

DISEASE:
Defects in FGG are a cause of congenital afibrinogenemia (CAFBN) [MIM:202400]. This rare autosomal recessive disorder is characterized by bleeding that varies from mild to severe and by complete absence or extremely low levels of plasma and platelet fibrinogen. Note=Patients with congenital fibrinogen abnormalities can manifest different clinical pictures. Some cases are clinically silent, some show a tendency toward bleeding and some show a predisposition for thrombosis with or without bleeding.

Similarity:
Contains 1 fibrinogen C-terminal domain.

Gene ID:
2266

Database links:
Entrez Gene: 2266 Human
Entrez Gene: 99571 Mouse
Omim: 134850 Human
SwissProt: P02679 Human
SwissProt: Q8VCM7 Mouse
Unigene: 16422 Mouse

Fibrinogen gamma chain,纤维蛋白原γ链抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.