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Factor VIII Monoclonal Antibody

商品貨號： PLA004795

適應性： 人

￥600元

規(guī)格：

40μL 100μL 200μL

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MSDS

說明書

商品描述

發(fā)貨日期： 7
基因名稱： F8
蛋白名稱： Coagulation factor VIII
Human_gene_id： 2157
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=2157
Human_swiss_prot_no： P00451
Human_swiss_link： http://www.uniprot.org/uniprotkb/P00451/entry
Mouse_swiss_prot_no： Q06194
Mouse_swiss_link： http://www.uniprot.org/uniprot/Q06194
特異性： Factor VIII Monoclonal Antibody detects endogenous levels of Factor VIII protein.
組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
來源： Monoclonal, Mouse
稀釋： WB 1:500 - 1:2000. ELISA: 1:10000. Not yet tested in other applications.
純化工藝： Affinity purification
儲存： -15°C to -25°C/1 year(Do not lower than -25°C)
說明書： 1
Msds： MSDS_Antibody.pdf
其他名稱： F8; F8C; Coagulation factor VIII; Antihemophilic factor; AHF; Procoagulant component
信號通路： Complement and coagulation cascades;
功能： disease:Defects in F8 are the cause of hemophilia A (HEMA) [MIM:306700]. HEMA is a common recessive X-linked coagulation disorder. The frequency of hemophilia A is 1-2 in 10,000 male births in all ethnic groups. About 50% of patients have severe hemophilia A with F8C activity less than 1% of normal; they have frequent spontaneous bleeding into joints, muscles and internal organs. Moderately severe hemophilia A occurs in about 10% of patients; F8C activity is 2-5% of normal, and there is bleeding after minor trauma. Mild hemophilia A, which occurs in 30-40% of patients, is associated with F8C activity of 5-30% and bleeding occurs only after significant trauma or surgery. Of particular interest for the understanding of the function of F8C is the category of CRM (cross-reacting material) positive patients (approximately 5%) that have considerable amount of F8C in their plasma (at least 30% of normal), but the protein is non-functional; i.e., the F8C activity is much less than the plasma protein level. CRM-reduced is another category of patients in which the F8C antigen and activity are reduced to approximately the same level. Most mutations are CRM negative, and probably affect the folding and stability of the protein.,domain:Domain F5/8 type C 2 is responsible for phospholipid-binding and essential for factor VIII activity.,function:Factor VIII, along with calcium and phospholipid, acts as a cofactor for factor IXa when it converts factor X to the activated form, factor Xa.,mass spectrometry:Disulfated PubMed:10368977,mass spectrometry:Monosulfated PubMed:10368977,mass spectrometry:Nonsulfated PubMed:10368977,mass spectrometry:Sulfated PubMed:10368977,mass spectrometry:Trisulfated PubMed:10368977,online information:Factor VIII entry,online information:Factor VIII mutation db,pharmaceutical:Available under the names Kogenate (Bayer) and Recombinate (Baxter and American Home Products). Used to treat hemophilia A.,PTM:Sulfation on Tyr-1699 is essential for binding vWF.,similarity:Belongs to the multicopper oxidase family.,similarity:Contains 1 F5/8 type C domain.,similarity:Contains 2 F5/8 type C domains.,similarity:Contains 3 F5/8 type A domains.,similarity:Contains 6 plastocyanin-like domains.,subunit:Interacts with vWF. vWF binding is essential for the stabilization of F8 in circulation.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細胞定位： Secreted, extracellular space.
組織表達： Brain,Hippocampus,Kidney,Plasma,
科研貨號： PLA004795

Factor VIII Monoclonal Antibody

Catalog No PLA004795

Product information

發(fā)貨日期： 7
基因名稱： F8
蛋白名稱： Coagulation factor VIII
Human_gene_id： 2157
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=2157
Human_swiss_prot_no： P00451
Human_swiss_link： http://www.uniprot.org/uniprotkb/P00451/entry
Mouse_swiss_prot_no： Q06194
Mouse_swiss_link： http://www.uniprot.org/uniprot/Q06194
特異性： Factor VIII Monoclonal Antibody detects endogenous levels of Factor VIII protein.
組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
來源： Monoclonal, Mouse
稀釋： WB 1:500 - 1:2000. ELISA: 1:10000. Not yet tested in other applications.
純化工藝： Affinity purification
儲存： -15°C to -25°C/1 year(Do not lower than -25°C)
說明書： 1
Msds： MSDS_Antibody.pdf
其他名稱： F8; F8C; Coagulation factor VIII; Antihemophilic factor; AHF; Procoagulant component
信號通路： Complement and coagulation cascades;
功能： disease:Defects in F8 are the cause of hemophilia A (HEMA) [MIM:306700]. HEMA is a common recessive X-linked coagulation disorder. The frequency of hemophilia A is 1-2 in 10,000 male births in all ethnic groups. About 50% of patients have severe hemophilia A with F8C activity less than 1% of normal; they have frequent spontaneous bleeding into joints, muscles and internal organs. Moderately severe hemophilia A occurs in about 10% of patients; F8C activity is 2-5% of normal, and there is bleeding after minor trauma. Mild hemophilia A, which occurs in 30-40% of patients, is associated with F8C activity of 5-30% and bleeding occurs only after significant trauma or surgery. Of particular interest for the understanding of the function of F8C is the category of CRM (cross-reacting material) positive patients (approximately 5%) that have considerable amount of F8C in their plasma (at least 30% of normal), but the protein is non-functional; i.e., the F8C activity is much less than the plasma protein level. CRM-reduced is another category of patients in which the F8C antigen and activity are reduced to approximately the same level. Most mutations are CRM negative, and probably affect the folding and stability of the protein.,domain:Domain F5/8 type C 2 is responsible for phospholipid-binding and essential for factor VIII activity.,function:Factor VIII, along with calcium and phospholipid, acts as a cofactor for factor IXa when it converts factor X to the activated form, factor Xa.,mass spectrometry:Disulfated PubMed:10368977,mass spectrometry:Monosulfated PubMed:10368977,mass spectrometry:Nonsulfated PubMed:10368977,mass spectrometry:Sulfated PubMed:10368977,mass spectrometry:Trisulfated PubMed:10368977,online information:Factor VIII entry,online information:Factor VIII mutation db,pharmaceutical:Available under the names Kogenate (Bayer) and Recombinate (Baxter and American Home Products). Used to treat hemophilia A.,PTM:Sulfation on Tyr-1699 is essential for binding vWF.,similarity:Belongs to the multicopper oxidase family.,similarity:Contains 1 F5/8 type C domain.,similarity:Contains 2 F5/8 type C domains.,similarity:Contains 3 F5/8 type A domains.,similarity:Contains 6 plastocyanin-like domains.,subunit:Interacts with vWF. vWF binding is essential for the stabilization of F8 in circulation.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細胞定位： Secreted, extracellular space.
組織表達： Brain,Hippocampus,Kidney,Plasma,
科研貨號： PLA004795

Hunan UPT Biotechnology Co.,Ltd

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E-mail：service@uptbio.com

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