Factor VIII Plasma | F8 plasma

Factor VIII Deficient Plasma

Cat. Num. AAA514010

• Gene Names: F8; AHF; F8B; F8C; HEMA; FVIII; DXS1253E
• Synonyms: Factor VIII; Factor VIII Deficient Plasma; F8 plasma

• Sequence Length: 2351

• Presentation: Frozen Factor VIII deficient plasma.
• Preparation/Handling: Thaw 1 ml vials in 37°C waterbath for 5 minutes; for bulk volumes, thawing time will be dependent on bottle size.
• Specifications: Lot specific assay results;; ; Assay:; PT (Stago Neoplastine C1 +): 12.6 sec; APTT (HemoslL APTT-SP): 80.1 sec; Fibrinogen (clottable): 3.22 g/L; FII (activity): 1.09 U/ml; FV (activity): 1.00 U/ml; FVII (activity): 0.90 U/ml; FVIII (activity): <0.01 U/ml; FIX (activity): 1.06 U/ml; FX (activity): 1.08 U/ml; FXI (activity): 0.70 U/ml; FXII (activity): 1.14 U/ml; FVIII Inhibitor assay (1:1, 120 min @ 37 degree C): Negative
• Note: Although this material was prepared with plasma collected from donors screened for CJD and which was tested at source and found negative for HBsAg, syphilis and antibodies to HIV and HeV an.d non-reactilleJof HIV-1 r.NA and HCV rNA by FDA approved tests, it should be har:1dled by personnel trained in the proper procedures for handling potential viral contaminants.
• Preparation and Storage: Plasma is shipped frozen and should be stored below -60°C. Product is stable until date stated on vial label when stored at -60°C. Once thawed, plasma is stable for 4 hours at 2-8°C in original vial.; Shipping: Dry Ice

Related Product Information for F8 plasma

Pooled normal citrated human plasma depleted of Factor VIII using antibodies directed to Factor VIII immobilized on agarose beads. Plasma contains 20mM HEPES.

Product Categories/Family for F8 plasma

Neutralizing Antibodies & Control IgG

NCBI and Uniprot Product Information

• NCBI GI #: 182803
• NCBI GeneID: 2157
• NCBI Official Full Name: factor VIII
• NCBI Official Synonym Full Names: coagulation factor VIII
• NCBI Official Symbol: F8
• NCBI Official Synonym Symbols: AHF; F8B; F8C; HEMA; FVIII; DXS1253E
• NCBI Protein Information: coagulation factor VIII
• UniProt Protein Name: Coagulation factor VIII
• Protein Family: Factor VIII intron 22 protein
• UniProt Gene Name: F8
• UniProt Synonym Gene Names: F8C; AHF

NCBI Description

This gene encodes coagulation factor VIII, which participates in the intrinsic pathway of blood coagulation; factor VIII is a cofactor for factor IXa which, in the presence of Ca+2 and phospholipids, converts factor X to the activated form Xa. This gene produces two alternatively spliced transcripts. Transcript variant 1 encodes a large glycoprotein, isoform a, which circulates in plasma and associates with von Willebrand factor in a noncovalent complex. This protein undergoes multiple cleavage events. Transcript variant 2 encodes a putative small protein, isoform b, which consists primarily of the phospholipid binding domain of factor VIIIc. This binding domain is essential for coagulant activity. Defects in this gene results in hemophilia A, a common recessive X-linked coagulation disorder. [provided by RefSeq, Jul 2008]

Uniprot Description

F8: 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. Defects in F8 are the cause of hemophilia A (HEMA). A disorder of blood coagulation characterized by a permanent tendency to hemorrhage. About 50% of patients have severe hemophilia resulting in frequent spontaneous bleeding into joints, muscles and internal organs. Less severe forms are characterized by bleeding after trauma or surgery. Of particular interest for the understanding of the function of F8 is the category of CRM (cross-reacting material) positive patients (approximately 5%) that have considerable amount of F8 in their plasma (at least 30% of normal), but the protein is non- functional; i.e. the F8 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. Belongs to the multicopper oxidase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: Xq28

Cellular Component: endoplasmic reticulum lumen; ER to Golgi transport vesicle; ER-Golgi intermediate compartment membrane; extracellular region; plasma membrane

Molecular Function: protein binding

Biological Process: blood coagulation; blood coagulation, intrinsic pathway; COPII coating of Golgi vesicle; ER to Golgi vesicle-mediated transport; platelet degranulation

Disease: Hemophilia A