维生素K依赖的蛋白C轻链抗体

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英文名称  Anti-protein C light chain

中文名称  维生素K依赖的蛋白C轻链抗体

别    名  Protein C; Anticoagulant protein C; Autoprothrombin IIA; Blood coagulation factor XIV; EC 3.4.21.69; PC; PROC; PROC1; Vitamin K dependent protein C precursor; APC; EC 3.4.21.69; PC; proC; PROC_HUMAN; Protein C (inactivator of coagulation factors Va and VIIIa); Vitamin K dependent protein C; Vitamin K-dependent protein C; Vitamin K-dependent protein C light chain.

浓    度  1mg/1ml

规 格  0.2ml/200μg

抗体来源  Rabbit  

克隆类型  polyclonal

交叉反应  Human, Rat

产品类型  一抗    

研究领域  心血管 细胞生物 免疫学 信号转导  

蛋白分子量  predicted molecular weight: 17/46kDa

性    状  Lyophilized or Liquid

维生素K依赖的蛋白C轻链抗体的主要功能是与抗原（包括外来的和自身的）相结合，从而有效地清除侵入机体内的微生物、寄生虫等异物，抗体（antibody）是一种应答抗原产生的、可与抗原特异性结合的蛋白质。每种抗体与特定的抗原决定基结合。这种结合可以使抗原失活，也可能无效但有时也会对机体造成病理性损害，如抗核抗体、抗双链DNA抗体、抗甲状腺球蛋白抗体等一些自身抗体的产生，对人体可造成危害。

免 疫 原  KLH conjugated synthetic peptide derived from human Vitamin K-dependent protein C light chain

亚    型  IgG

纯化方法  affinity purified by Protein A

储 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  ICC=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.  

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.

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

产品介绍 This gene encodes a vitamin K-dependent plasma glycoprotein. The encoded protein is cleaved to its activated form by the thrombin-thrombomodulin complex. This activated form contains a serine protease domain and functions in degradation of the activated forms of coagulation factors V and VIII. Mutations in this gene have been associated with thrombophilia due to protein C deficiency, neonatal purpura fulminans, and recurrent venous thrombosis.[provided by RefSeq, Dec 2009].

Function : Protein C is a vitamin K-dependent serine protease that regulates blood coagulation by inactivating factors Va and VIIIa in the presence of calcium ions and phospholipids.

Subunit : Synthesized as a single chain precursor, which is cleaved into a light chain and a heavy chain held together by a disulfide bond. The enzyme is then activated by thrombin, which cleaves a tetradecapeptide from the amino end of the heavy chain; this reaction, which occurs at the surface of endothelial cells, is strongly promoted by thrombomodulin.

Tissue Specificity : Plasma; synthesized in the liver.

Post-translational modifications : The vitamin K-dependent, enzymatic carboxylation of some Glu residues allows the modified protein to bind calcium.

N- and O-glycosylated. Partial (70%) N-glycosylation of Asn-371 with an atypical N-X-C site produces a higher molecular weight form referred to as alpha. The lower molecular weight form, not N-glycosylated at Asn-371, is beta. O-glycosylated with core 1 or possibly core 8 glycans.

The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.

May be phosphorylated on a Ser or Thr in a region (AA 25-30) of the propeptide.

DISEASE : Defects in PROC are the cause of thrombophilia due to protein C deficiency, autosomal dominant (THPH3) [MIM:176860]. A hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. However, many adults with heterozygous disease may be asymptomatic. Individuals with decreased amounts of protein C are classically referred to as having type I protein C deficiency and those with normal amounts of a functionally defective protein as having type II deficiency.

Defects in PROC are the cause of thrombophilia due to protein C deficiency, autosomal recessive (THPH4) [MIM:612304]. A hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. It results in a thrombotic condition that can manifest as a severe neonatal disorder or as a milder disorder with late-onset thrombophilia. The severe form leads to neonatal death through massive neonatal venous thrombosis. Often associated with ecchymotic skin lesions which can turn necrotic called purpura fulminans, this disorder is very rare.

Similarity : Belongs to the peptidase S1 family.

Contains 2 EGF-like domains.

维生素K依赖的蛋白C轻链抗体Contains 1 Gla (gamma-carboxy-glutamate) domain.

Contains 1 peptidase S1 domain.

Database links : NCBI Reference Sequence: NP_000303

UniProtKB/Swiss-Prot: P04070