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15034 details
Primary information
ID15034
Therapeutic IDTh1563
Protein NameVon Willebrand factor human
Sequence>Th1563_Von_Willebrand_factor_human MIPARFAGVLLALALILPGTLCAEGTRGRSSTARCSLFGSDFVNTFDGSMYSFAGYCSYLAGGCQKRSFSIIGDFQNGKRVSLSVYLGEFFDIHLFVNGTVTQGDQRVSMPYASKGLYLETEAGYYKLSGEAYGFVARIDGSGNFQVLLSDRYFNKTCGLCGNFNIFAEDDFMTQEGTLTSDPYDFANSWALSSGEQWCERASPPSSSCNISSGEMQKGLWEQCQLLKSTSVFARCHPLVDPEPFVALCEKTLCECAGGLECACPALLEYARTCAQEGMVLYGWTDHSACSPVCPAGMEYRQCVSPCARTCQSLHINEMCQERCVDGCSCPEGQLLDEGLCVESTECPCVHSGKRYPPGTSLSRDCNTCICRNSQWICSNEECPGECLVTGQSHFKSFDNRYFTFSGICQYLLARDCQDHSFSIVIETVQCADDRDAVCTRSVTVRLPGLHNSLVKLKHGAGVAMDGQDIQLPLLKGDLRIQHTVTASVRLSYGEDLQMDWDGRGRLLVKLSPVYAGKTCGLCGNYNGNQGDDFLTPSGLAEPRVEDFGNAWKLHGDCQDLQKQHSDPCALNPRMTRFSEEACAVLTSPTFEACHRAVSPLPYLRNCRYDVCSCSDGRECLCGALASYAAACAGRGVRVAWREPGRCELNCPKGQVYLQCGTPCNLTCRSLSYPDEECNEACLEGCFCPPGLYMDERGDCVPKAQCPCYYDGEIFQPEDIFSDHHTMCYCEDGFMHCTMSGVPGSLLPDAVLSSPLSHRSKRSLSCRPPMVKLVCPADNLRAEGLECTKTCQNYDLECMSMGCVSGCLCPPGMVRHENRCVALERCPCFHQGKEYAPGETVKIGCNTCVCRDRKWNCTDHVCDATCSTIGMAHYLTFDGLKYLFPGECQYVLVQDYCGSNPGTFRILVGNKGCSHPSVKCKKRVTILVEGGEIELFDGEVNVKRPMKDETHFEVVESGRYIILLLGKALSVVWDRHLSISVVLKQTYQEKVCGLCGNFDGIQNNDLTSSNLQVEEDPVDFGNSWKVSSQCADTRKVPLDSSPATCHNNIMKQTMVDSSCRILTSDVFQDCNKLVDPEPYLDVCIYDTCSCESIGDCACFCDTIAAYAHVCAQHGKVVTWRTATLCPQSCEERNLRENGYECEWRYNSCAPACQVTCQHPEPLACPVQCVEGCHAHCPPGKILDELLQTCVDPEDCPVCEVAGRRFASGKKVTLNPSDPEHCQICHCDVVNLTCEACQEPGGLVVPPTDAPVSPTTLYVEDISEPPLHDFYCSRLLDLVFLLDGSSRLSEAEFEVLKAFVVDMMERLRISQKWVRVAVVEYHDGSHAYIGLKDRKRPSELRRIASQVKYAGSQVASTSEVLKYTLFQIFSKIDRPEASRIALLLMASQEPQRMSRNFVRYVQGLKKKKVIVIPVGIGPHANLKQIRLIEKQAPENKAFVLSSVDELEQQRDEIVSYLCDLAPEAPPPTLPPHMAQVTVGPGLLGVSTLGPKRNSMVLDVAFVLEGSDKIGEADFNRSKEFMEEVIQRMDVGQDSIHVTVLQYSYMVTVEYPFSEAQSKGDILQRVREIRYQGGNRTNTGLALRYLSDHSFLVSQGDREQAPNLVYMVTGNPASDEIKRLPGDIQVVPIGVGPNANVQELERIGWPNAPILIQDFETLPREAPDLVLQRCCSGEGLQIPTLSPAPDCSQPLDVILLLDGSSSFPASYFDEMKSFAKAFISKANIGPRLTQVSVLQYGSITTIDVPWNVVPEKAHLLSLVDVMQREGGPSQIGDALGFAVRYLTSEMHGARPGASKAVVILVTDVSVDSVDAAADAARSNRVTVFPIGIGDRYDAAQLRILAGPAGDSNVVKLQRIEDLPTMVTLGNSFLHKLCSGFVRICMDEDGNEKRPGDVWTLPDQCHTVTCQPDGQTLLKSHRVNCDRGLRPSCPNSQSPVKVEETCGCRWTCPCVCTGSSTRHIVTFDGQNFKLTGSCSYVLFQNKEQDLEVILHNGACSPGARQGCMKSIEVKHSALSVELHSDMEVTVNGRLVSVPYVGGNMEVNVYGAIMHEVRFNHLGHIFTFTPQNNEFQLQLSPKTFASKTYGLCGICDENGANDFMLRDGTVTTDWKTLVQEWTVQRPGQTCQPILEEQCLVPDSSHCQVLLLPLFAECHKVLAPATFYAICQQDSCHQEQVCEVIASYAHLCRTNGVCVDWRTPDFCAMSCPPSLVYNHCEHGCPRHCDGNVSSCGDHPSEGCFCPPDKVMLEGSCVPEEACTQCIGEDGVQHQFLEAWVPDHQPCQICTCLSGRKVNCTTQPCPTAKAPTCGLCEVARLRQNADQCCPEYECVCDPVSCDLPPVPHCERGLQPTLTNPGECRPNFTCACRKEECKRVSPPSCPPHRLPTLRKTQCCDEYECACNCVNSTVSCPLGYLASTATNDCGCTTTTCLPDKVCVHRSTIYPVGQFWEEGCDVCTCTDMEDAVMGLRVAQCSQKPCEDSCRSGFTYVLHEGECCGRCLPSACEVVTGSPRGDSQSSWKSVGSQWASPENPCLINECVRVKEEVFIQQRNVSCPQLEVPVCPSGFQLSCKTSACCPSCRCERMEACMLNGTVIGPGKTVMIDVCTTCRCMVQVGVISGFKLECRKTTCNPCPLGYKEENNTGECCGRCLPTACTIQLRGGQIMTLKRDETLQDGCDTHFCKVNERGEYFWEKRVTGCPPFDEHKCLAEGGKIMKIPGTCCDTCEEPECNDITARLQYVKVGSCKSEVEVDIHYCQGKCASKAMYSIDINDVQDQCSCCSPTRTEPMQVALHCTNGSVVYHEVLNAMECKCSPRKCSK
Molecular Weight20000000
Chemical FormulaNA
Isoelectric Point5.7-5.9
HydrophobicityNA
Melting pointNA
Half-lifeThe reported terminal half-life of the concentrate of vWF and factor VIII is of 15.8 hours.[A32279]
Descriptionvon Willebrand Factor (vWF) is a multimeric glycoprotein that consists of disulfide-bridge linked dimers. It is usually circulating in plasma as a stable complex with the coagulation factor VIII. The human vWF, as a drug product, is a complex that also contains the coagulation factor VIII.[A32268] This complex was developed by Octapharma Pharmazeutika Produktionsges and FDA approved on August 21, 2015.[L1878]
Indication/DiseaseThe vWF and Factor VIII complex is indicated for the prevention of excessive bleeding during and after minor and major surgery in adult and pediatric von Willebrand disease patients. It is also indicated for the on-demand treatment and control of bleeding episodes.[L1880] The von Willebrand disease is an inherited disorder characterized by the deficiency or misfunction of the von Willebrand factor (vWF). Due to this deficiency, the blood cannot clot properly and the patients that present this disease are prone to prolonged or excessive bleeding. There are three types of this disease, and type 3 is an autosomal recessive inherited disorder marked by very low or absent levels of vWF.[L1867]
PharmacodynamicsThe efficacy in clinical trials of vWF in surgical procedures is of 96.7% regardless the type of von Willebrand disease.[L1880] In a perioperative setting, the hemostatic effects were rated as excellent in a dose of 848.6 IU/kg and no anti-factor VII or inhibitors were detected.[A32270] These excellent results come from the re-establishment of platelet-adhesion to the subendothelium at sites of vascular damage and the aggregation of platelets. vWF allows the protection of factor VIII from the rapid degradation and thus it restores normal coagulation.[L1880]
Mechanism of ActionThe vWF is involved in primary and secondary homeostasis and it is also a carrier and stabilizing protein that protects the coagulation factor VIII from proteolysis and clearance.[A32268] vWF also presents an adhesive function in which it mediates the binding between platelets and subendothelial tissues.[A32289] The external human vWF acts as the endogenous vWF and thus, it presents all the functions abovementioned.[L1880]
ToxicityThe administration of human vWF seems to be very well accepted.
MetabolismThe endogenous regulator of the vWF is ADAMTS13. The interaction between these two proteins results in the proteolysis of vWF. This proteolysis occurs primarily in the cleavage site at the domain A2 which is a target domain for ADAMTS13.[A32290]
AbsorptionAccording to clinical trials in patients with von Willebrand disease, the AUC, Cmax and mean residence time was approximately 1119 h.IU/dL, 76 IU/dL and 20.6 hours, respectively.[A32270]
The volume of distribution of the human concentrate vWF is 69.7 ml/kg.[A32270]
ClearanceThe clearance rate of the human concentrate vWF is 3.9 ml.h/kg.[A32270]
CategoriesProteins
Patents NumberNA
Date of IssueNA
Date of ExpiryNA
Drug InteractionNA
TargetCoagulation factor VIII,Collagen alpha-1(I) chain
Brand NameWilate - von Willebrand Factor/Coagulation Factor VIII Complex (Human)
CompanyOctapharma Pharmazeutika Produktionsgesellschaft M.B.H.
Brand DescriptionOctapharma Pharmazeutika Produktionsgesellschaft M.B.H.
Prescribed ForIntravenous
Chemical NameNA
FormulationWILATE is contraindicated in patients with known hypersensitivity reactions, including anaphylactic or severe systemic reactions, to human plasma-derived products, any ingredient in the formulation [see DESCRIPTION], or components of the container.
Physical Appearance The most common adverse reactions to treatment with WILATE ( ≥ 1%) in patients with VWD were hypersensitivity reactions, urticaria, and dizziness.
Route of AdministrationWILATE is a human plasma-derived, sterile, purified, double virus inactivated von Willebrand Factor/Coagulation Factor VIII Complex. WILATE is supplied as a lyophilized powder for reconstitution for intravenous injection. The diluent for reconstitution of the lyophilized powder is Water for Injection with 0.1% Polysorbate 80.
Recommended DosageWILATE is indicated in children and adults with von Willebrand disease for: On-demand treatment and control of bleeding episodes Perioperative management of bleeding
ContraindicationNA
Side EffectsNA
Useful Link 1Link
Useful Link 2Link
RemarksNA