Identification

Summary

Von Willebrand factor human is a blood clotting factor used to manage bleeding episodes in von Willebrand disease and hemophilia A,.

Brand Names
Vonvendi, Wilate
Generic Name
Von Willebrand factor human
DrugBank Accession Number
DB13133
Background

The human von Willebrand factor (vWF) is a human plasma-derived vWF, an endogenous large multimeric plasma glycoprotein involved in hemostasis. It serves a dual role in hemostasis by mediating platelet adhesion and aggregation at the site of blood vessel injury and stabilizing procoagulant factor VIII (FVIII).4 Exogenous sources of vWF are used to restore functional levels of vWF in blood disorders associated with deficient or abnormal blood clotting. The human vWF is used to manage and control bleeding episodes in patients with von Willebrand disease and hemophilia A. It was first approved by the FDA in 2015.7 As vWF is normally present in the blood as a stable complex with coagulation factor III, therapeutic vWF products are also available as a combination product with antihemophilic factor human.6 A recombinant form of vWF, vonicog alfa, is also available to enhance production and avoid the theoretical risk of pathogen transmission from plasma donors.4

Type
Biotech
Groups
Approved, Investigational
Biologic Classification
Protein Based Therapies
Blood factors
Protein Structure
Protein Chemical Formula
Not Available
Protein Average Weight
Not Available
Sequences
>Isoform 1
MIPARFAGVLLALALILPGTLCAEGTRGRSSTARCSLFGSDFVNTFDGSMYSFAGYCSYL
LAGGCQKRSFSIIGDFQNGKRVSLSVYLGEFFDIHLFVNGTVTQGDQRVSMPYASKGLYL
ETEAGYYKLSGEAYGFVARIDGSGNFQVLLSDRYFNKTCGLCGNFNIFAEDDFMTQEGTL
TSDPYDFANSWALSSGEQWCERASPPSSSCNISSGEMQKGLWEQCQLLKSTSVFARCHPL
VDPEPFVALCEKTLCECAGGLECACPALLEYARTCAQEGMVLYGWTDHSACSPVCPAGME
YRQCVSPCARTCQSLHINEMCQERCVDGCSCPEGQLLDEGLCVESTECPCVHSGKRYPPG
TSLSRDCNTCICRNSQWICSNEECPGECLVTGQSHFKSFDNRYFTFSGICQYLLARDCQD
HSFSIVIETVQCADDRDAVCTRSVTVRLPGLHNSLVKLKHGAGVAMDGQDVQLPLLKGDL
RIQHTVTASVRLSYGEDLQMDWDGRGRLLVKLSPVYAGKTCGLCGNYNGNQGDDFLTPSG
LAEPRVEDFGNAWKLHGDCQDLQKQHSDPCALNPRMTRFSEEACAVLTSPTFEACHRAVS
PLPYLRNCRYDVCSCSDGRECLCGALASYAAACAGRGVRVAWREPGRCELNCPKGQVYLQ
CGTPCNLTCRSLSYPDEECNEACLEGCFCPPGLYMDERGDCVPKAQCPCYYDGEIFQPED
IFSDHHTMCYCEDGFMHCTMSGVPGSLLPDAVLSSPLSHRSKRSLSCRPPMVKLVCPADN
LRAEGLECTKTCQNYDLECMSMGCVSGCLCPPGMVRHENRCVALERCPCFHQGKEYAPGE
TVKIGCNTCVCQDRKWNCTDHVCDATCSTIGMAHYLTFDGLKYLFPGECQYVLVQDYCGS
NPGTFRILVGNKGCSHPSVKCKKRVTILVEGGEIELFDGEVNVKRPMKDETHFEVVESGR
YIILLLGKALSVVWDRHLSISVVLKQTYQEKVCGLCGNFDGIQNNDLTSSNLQVEEDPVD
FGNSWKVSSQCADTRKVPLDSSPATCHNNIMKQTMVDSSCRILTSDVFQDCNKLVDPEPY
LDVCIYDTCSCESIGDCACFCDTIAAYAHVCAQHGKVVTWRTATLCPQSCEERNLRENGY
ECEWRYNSCAPACQVTCQHPEPLACPVQCVEGCHAHCPPGKILDELLQTCVDPEDCPVCE
VAGRRFASGKKVTLNPSDPEHCQICHCDVVNLTCEACQEPGGLVVPPTDAPVSPTTLYVE
DISEPPLHDFYCSRLLDLVFLLDGSSRLSEAEFEVLKAFVVDMMERLRISQKWVRVAVVE
YHDGSHAYIGLKDRKRPSELRRIASQVKYAGSQVASTSEVLKYTLFQIFSKIDRPEASRI
TLLLMASQEPQRMSRNFVRYVQGLKKKKVIVIPVGIGPHANLKQIRLIEKQAPENKAFVL
SSVDELEQQRDEIVSYLCDLAPEAPPPTLPPDMAQVTVGPGLLGVSTLGPKRNSMVLDVA
FVLEGSDKIGEADFNRSKEFMEEVIQRMDVGQDSIHVTVLQYSYMVTVEYPFSEAQSKGD
ILQRVREIRYQGGNRTNTGLALRYLSDHSFLVSQGDREQAPNLVYMVTGNPASDEIKRLP
GDIQVVPIGVGPNANVQELERIGWPNAPILIQDFETLPREAPDLVLQRCCSGEGLQIPTL
SPAPDCSQPLDVILLLDGSSSFPASYFDEMKSFAKAFISKANIGPRLTQVSVLQYGSITT
IDVPWNVVPEKAHLLSLVDVMQREGGPSQIGDALGFAVRYLTSEMHGARPGASKAVVILV
TDVSVDSVDAAADAARSNRVTVFPIGIGDRYDAAQLRILAGPAGDSNVVKLQRIEDLPTM
VTLGNSFLHKLCSGFVRICMDEDGNEKRPGDVWTLPDQCHTVTCQPDGQTLLKSHRVNCD
RGLRPSCPNSQSPVKVEETCGCRWTCPCVCTGSSTRHIVTFDGQNFKLTGSCSYVLFQNK
EQDLEVILHNGACSPGARQGCMKSIEVKHSALSVELHSDMEVTVNGRLVSVPYVGGNMEV
NVYGAIMHEVRFNHLGHIFTFTPQNNEFQLQLSPKTFASKTYGLCGICDENGANDFMLRD
GTVTTDWKTLVQEWTVQRPGQTCQPILEEQCLVPDSSHCQVLLLPLFAECHKVLAPATFY
AICQQDSCHQEQVCEVIASYAHLCRTNGVCVDWRTPDFCAMSCPPSLVYNHCEHGCPRHC
DGNVSSCGDHPSEGCFCPPDKVMLEGSCVPEEACTQCIGEDGVQHQFLEAWVPDHQPCQI
CTCLSGRKVNCTTQPCPTAKAPTCGLCEVARLRQNADQCCPEYECVCDPVSCDLPPVPHC
ERGLQPTLTNPGECRPNFTCACRKEECKRVSPPSCPPHRLPTLRKTQCCDEYECACNCVN
STVSCPLGYLASTATNDCGCTTTTCLPDKVCVHRSTIYPVGQFWEEGCDVCTCTDMEDAV
MGLRVAQCSQKPCEDSCRSGFTYVLHEGECCGRCLPSACEVVTGSPRGDSQSSWKSVGSQ
WASPENPCLINECVRVKEEVFIQQRNVSCPQLEVPVCPSGFQLSCKTSACCPSCRCERME
ACMLNGTVIGPGKTVMIDVCTTCRCMVQVGVISGFKLECRKTTCNPCPLGYKEENNTGEC
CGRCLPTACTIQLRGGQIMTLKRDETLQDGCDTHFCKVNERGEYFWEKRVTGCPPFDEHK
CLAEGGKIMKIPGTCCDTCEEPECNDITARLQYVKVGSCKSEVEVDIHYCQGKCASKAMY
SIDINDVQDQCSCCSPTRTEPMQVALHCTNGSVVYHEVLNAMECKCSPRKCSK
>Isoform 2
MGAQDEEEGIQDLDGLLVFDKIVEVTLLNLPWYNEETEGQRGEMTAPKSPRAKIRGTLCA
EGTRGRSSTARCSLFGSDFVNTFDGSMYSFAGYCSYLLAGGCQKRSFSIIGDFQNGKRVS
LSVYLGEFFDIHLFVNGTVTQGDQRVSMPYASKGLYLETEAGYYKLSGEAYGFVARIDGS
GNFQVLLSDRYFNKTCGLCGNFNIFAEDDFMTQEGTLTSDPYDFANSWALSSGEQWCERA
SPPSSSCNISSGEMQKEEPECNDITARLQYVKVGSCKSEVEVDIHYCQGKCASKAMYSID
INDVQDQCSCCSPTRTEPMQVALHCTNGSVVYHEVLNAMECKCSPRKCSKI
References:
  1. UniProt: UniProtKB - P04275 (VWF_HUMAN) [Link]
Download FASTA Format
Synonyms
  • Factor con Willebrand
  • Factor von Willebrand
  • Human von willebrand factor
  • Von willebrand factor
  • Von willebrand factor (human)
  • Von willebrand factor complex (human)
  • von Willebrand factor, human
  • Von willebrand's factor

Pharmacology

Indication

The von Willebrand factor (vWF) is indicated to manage and control bleeding episodes in adults with von Willebrand disease. It is also used for perioperative management of bleeding in these patients. It is also used for routine prophylaxis to reduce the frequency of bleeding episodes in patients with severe Type 3 von Willebrand disease receiving on-demand therapy.7

In combination with antihemophilic factor human, vWF is also used to manage and control bleeding associated with von Willebrand disease in children and adults, as well as for perioperative management of bleeding in these patients. This combination product is also used for the management and control of bleeding episodes in adolescents and adults with hemophilia A, as well as for routine prophylaxis to reduce the frequency of bleeding episodes.6

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Associated Conditions
Associated Therapies
Contraindications & Blackbox Warnings
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Pharmacodynamics

von Willebrand factor (vWF) promotes hemostasis. The binding capacity and affinity of exogenous human vWF to factor VIII in plasma is comparable to that of endogenous vWF, allowing for vWF to reduce factor VIII clearance. In patients with type 3 von Willebrand disease, a single infusion of human vWF led to an increase of factor VIII coagulation activity with peak levels observed approximately 24 hours post-infusion.7 The efficacy in clinical trials of vWF in surgical procedures is 96.7% regardless of the type of von Willebrand disease.[L1880]

Mechanism of action

Clotting factor VIII or coagulation factor VIII is a glycoprotein involved in the intrinsic pathway of the blood coagulation cascade.6 It normally circulates in human plasma in a stable complex with von Willebrand factor (vWF), which is a multimeric glycoprotein. vWF acts as a carrier and stabilizing protein for coagulation factor VIII, as it is prone to rapid proteolysis and clearance from plasma.1,7 vWF consists of disulfide bridge linked dimers of the 225-kDa single-chain molecule,1 with the large multimer of vWF being the most effective in supporting adhesion between platelet and collagen or platelet receptors.7 vWF in circulation functions as a molecular carrier for various proteins other than coagulation factor VIII, such as osteoprotegerin and galectins and recruits platelets upon vascular injury 3 to promote platelet adhesion to the damaged vascular sub-endothelial matrix and platelet aggregation.3,7

Both coagulation factor VIII and vWF are essential blood clotting factors in normal hemostasis; however, certain blood disorders such as hemophilia A and von Willebrand disease are associated with reduced or deficient levels of functional clotting factors. Reduced levels of vWF in plasma lead to decreased levels and activity of factor VIII, and abnormal platelet function, thereby resulting in excessive bleeding.6 Exogenous sources of human vWD aim to restore the levels of vWF in circulation to control and prevent bleeding episodes in patients with the reduced capability of blood clotting. Exogenous vWF treatment is also available as a combination product also containing exogenous coagulation factor VIII to replenish the levels of both clotting factors.6

TargetActionsOrganism
ACoagulation factor VIII
carrier
stabilization
Humans
ACollagen alpha-1(I) chain
binder
Humans
UAsialoglycoprotein receptor 1
ligand
Humans
UProlow-density lipoprotein receptor-related protein 1Not AvailableHumans
Absorption

In patients with Type 3 von Willebrand disease who were previously treated on-demand with any vWF product prior to study entry, the mean (SD) Cmax of recombinant vWF at steady state was 86.4 (34.2) IU/dL. In patients who were previously treated prophylactically with a plasma-derived vWF product, the mean (SD) Cmax was 90.6 (33.7) IU/dL.7

Volume of distribution

The volume of distribution of the human concentrate vWF is 69.7 mL/kg.2

Protein binding

The vWF normally forms a complex with coagulation factor VIII in circulation.7

Metabolism

ADAMTS13, or von Willebrand factor-cleaving protease, is a disintegrin and metalloprotease that normally cleaves vWF.4 Proteolysis of vWF occurs primarily in the cleavage site at domain A2, which is a target domain for ADAMTS13.5

Route of elimination

As with endogenous vWF, exogenous sources of vWF are also expected to undergo elimination by the liver and spleen, which take up vWF as part of an active regulatory mechanism.3

Half-life

In patients with Type 3 von Willebrand disease who were previously treated on-demand with any vWF product prior to study entry, the mean (SD) half-life of recombinant vWF at steady state was 16.5 (4.13) hours. In patients who were previously treated prophylactically with a plasma-derived vWF product, the mean (SD) half-life was 14.1 (6.13) hours.7

Clearance

In patients with Type 3 von Willebrand disease who were previously treated on-demand with any vWF product prior to study entry, the mean (SD) clearance of recombinant vWF at steady state was 0.04 (0.012) (dL/kg)/h. In patients who were previously treated prophylactically with a plasma-derived vWF product, the mean (SD) clearance was 0.04 (0.014) (dL/kg)/h.7

Adverse Effects
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Toxicity

There is limited information regarding the LD50 and overdose of vWF.

Pathways
Not Available
Pharmacogenomic Effects/ADRs
Not Available

Interactions

Drug Interactions
This information should not be interpreted without the help of a healthcare provider. If you believe you are experiencing an interaction, contact a healthcare provider immediately. The absence of an interaction does not necessarily mean no interactions exist.
DrugInteraction
AbciximabThe therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Abciximab.
AcenocoumarolThe therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Acenocoumarol.
Alpha-1-proteinase inhibitorAlpha-1-proteinase inhibitor may increase the thrombogenic activities of Von Willebrand factor human.
AlteplaseThe therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Alteplase.
Aminocaproic acidThe risk or severity of adverse effects can be increased when Aminocaproic acid is combined with Von Willebrand factor human.
AncrodThe therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Ancrod.
AnistreplaseThe therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Anistreplase.
Antithrombin AlfaThe therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Antithrombin Alfa.
Antithrombin III humanThe therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Antithrombin III human.
ApixabanThe therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Apixaban.
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Food Interactions
No interactions found.

Products

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Brand Name Prescription Products
NameDosageStrengthRouteLabellerMarketing StartMarketing EndRegionImage
VonvendiKit1300 [iU]/10mLIntravenousBaxalta US Inc.2015-12-08Not applicableUS flag
VonvendiKit650 [iU]/5mLIntravenousBaxalta US Inc.2015-12-08Not applicableUS flag
Mixture Products
NameIngredientsDosageRouteLabellerMarketing StartMarketing EndRegionImage
AlphanateVon Willebrand factor human (2400 unit / vial) + Antihemophilic factor human (2000 unit / vial)Powder, for solutionIntravenousGrifols Biologicals LlcNot applicableNot applicableCanada flag
AlphanateVon Willebrand factor human (600 unit / vial) + Antihemophilic factor human (500 unit / vial)Powder, for solutionIntravenousGrifols Biologicals LlcNot applicableNot applicableCanada flag
AlphanateVon Willebrand factor human (1800 unit / vial) + Antihemophilic factor human (1500 unit / vial)Powder, for solutionIntravenousGrifols Biologicals LlcNot applicableNot applicableCanada flag
AlphanateVon Willebrand factor human (300 unit / vial) + Antihemophilic factor human (250 unit / vial)Powder, for solutionIntravenousGrifols Biologicals LlcNot applicableNot applicableCanada flag
AlphanateVon Willebrand factor human (1200 unit / vial) + Antihemophilic factor human (1000 unit / vial)Powder, for solutionIntravenousGrifols Biologicals LlcNot applicableNot applicableCanada flag
ALPHANATE FOR INJECTION 1000 iu/vialVon Willebrand factor human (>400 IU) + Beroctocog alfa (1000 IU)Injection, powder, for solutionIntravenousGRIFOLS ASIA PACIFIC PTE. LTD.1996-11-28Not applicable
ALPHANATE FOR INJECTION 250 iu/vialVon Willebrand factor human (>100 IU) + Beroctocog alfa (250 IU)Injection, powder, for solutionIntravenousGRIFOLS ASIA PACIFIC PTE. LTD.1996-11-28Not applicable
ALPHANATE FOR INJECTION 500 iu/vialVon Willebrand factor human (>200 IU) + Beroctocog alfa (500 IU)Injection, powder, for solutionIntravenousGRIFOLS ASIA PACIFIC PTE. LTD.1996-11-28Not applicable
BIOSTATE FOR INJECTION 50 iu/mlVon Willebrand factor human (100 iu/mL) + Antihemophilic factor human (50 iu/mL)Injection, powder, for solutionIntravenousBLOOD SERVICES GROUP, HEALTH SCIENCES AUTHORITY2003-01-31Not applicable
FANHDI 250 U.I.Von Willebrand factor human (300 IU) + Antihemophilic factor, human recombinant (250 IU)Injection, powder, lyophilized, for solutionIntravenousGRIFOLS BIOLOGICALS LLC.2008-06-192018-05-17Colombia flag

Categories

ATC Codes
B02BD06 — Von willebrand factor and coagulation factor viii in combinationB02BD10 — Von willebrand factor
Drug Categories
Chemical TaxonomyProvided by Classyfire
Description
Not Available
Kingdom
Organic Compounds
Super Class
Organic Acids
Class
Carboxylic Acids and Derivatives
Sub Class
Amino Acids, Peptides, and Analogues
Direct Parent
Peptides
Alternative Parents
Not Available
Substituents
Not Available
Molecular Framework
Not Available
External Descriptors
Not Available
Affected organisms
  • Humans

Chemical Identifiers

UNII
ZE22NE22F1
CAS number
109319-16-6

References

General References
  1. Clifton JG, Huang F, Kovac S, Yang X, Hixson DC, Josic D: Proteomic characterization of plasma-derived clotting factor VIII-von Willebrand factor concentrates. Electrophoresis. 2009 Oct;30(20):3636-46. doi: 10.1002/elps.200900270. [Article]
  2. Klukowska A, Windyga J, Batorova A: Clinical efficacy of a novel VWF-containing FVIII concentrate, Wilate((R)), in the prophylaxis and treatment of bleeding episodes in previously treated haemophilia A patients. Thromb Res. 2011 Mar;127(3):247-53. doi: 10.1016/j.thromres.2010.11.030. Epub 2011 Jan 8. [Article]
  3. Lenting PJ, Christophe OD, Denis CV: von Willebrand factor biosynthesis, secretion, and clearance: connecting the far ends. Blood. 2015 Mar 26;125(13):2019-28. doi: 10.1182/blood-2014-06-528406. Epub 2015 Feb 23. [Article]
  4. Gill JC, Castaman G, Windyga J, Kouides P, Ragni M, Leebeek FW, Obermann-Slupetzky O, Chapman M, Fritsch S, Pavlova BG, Presch I, Ewenstein B: Hemostatic efficacy, safety, and pharmacokinetics of a recombinant von Willebrand factor in severe von Willebrand disease. Blood. 2015 Oct 22;126(17):2038-46. doi: 10.1182/blood-2015-02-629873. Epub 2015 Aug 3. [Article]
  5. Chung MC, Popova TG, Jorgensen SC, Dong L, Chandhoke V, Bailey CL, Popov SG: Degradation of circulating von Willebrand factor and its regulator ADAMTS13 implicates secreted Bacillus anthracis metalloproteases in anthrax consumptive coagulopathy. J Biol Chem. 2008 Apr 11;283(15):9531-42. doi: 10.1074/jbc.M705871200. Epub 2008 Feb 8. [Article]
  6. FDA Approved Drug Products: WILATE [von Willebrand Factor/Coagulation Factor VIII Complex (Human)] Lyophilized Powder for Solution for Intravenous Injection [Link]
  7. FDA Approved Drug Products: VONVENDI [von Willebrand factor (recombinant)] lyophilized powder for solution, for intravenous injection [Link]
PubChem Substance
347911428
RxNav
826072
MSDS
Download (27 KB)

Clinical Trials

Clinical Trials
PhaseStatusPurposeConditionsCount
4CompletedTreatmentVon Willebrand's Disease1
4RecruitingTreatmentBleeding1
4TerminatedTreatmentHemophilia A With Inhibitors / Hemophilia As1
4TerminatedTreatmentHemophilia As1
3Active Not RecruitingTreatmentVon Willebrand's Disease2
3CompletedNot AvailableHemophilia As1
3CompletedPreventionVon Willebrand's Disease1
3CompletedTreatmentHemophilia As2
3CompletedTreatmentSevere Hemophilia A2
3CompletedTreatmentVon Willebrand's Disease2

Pharmacoeconomics

Manufacturers
Not Available
Packagers
Not Available
Dosage Forms
FormRouteStrength
InjectionIntravenous
Injection, powder, for solutionIntravenous1000 IU
Injection, powder, for solutionIntravenous250 IU
Injection, powder, for solutionIntravenous500 IU
Injection, powder, for solutionIntravenous50 iu/mL
Injection, powder, lyophilized, for solutionIntravenous1000 IU
Powder, for solutionParenteral
Injection, powder, for solutionIntravenous
Injection, powder, for solutionIntravenous
Injection, powder, for solutionIntravenous100 iu/ml
Injection, powder, lyophilized, for solutionIntravenous
Injection, powder, for solutionIntravenous1000 UI/2400UI
Injection, powder, for solutionIntravenous250 UI/600UI
Injection, powder, for solutionIntravenous500 UI/1200UI
PowderIntravenous
PowderIntravenous500 IU
Injection, powder, lyophilized, for solutionIntravenous500 IU
KitIntravenous1300 [iU]/10mL
KitIntravenous650 [iU]/5mL
Injection, powder, lyophilized, for solutionParenteral
Powder, for solutionIntravenous
Injection, powder, for solutionParenteral
Injection, powder, for solutionIntravenous100 UI/ML
Injection, powder, for solutionParenteral
Prices
Not Available
Patents
Not Available

Properties

State
Solid
Experimental Properties
PropertyValueSource
isoelectric point5.7-5.9Fulcher, et al. Blood. (1983).

Targets

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Kind
Protein
Organism
Humans
Pharmacological action
Yes
Actions
Carrier
Stabilization
General Function
Oxidoreductase activity
Specific 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.
Gene Name
F8
Uniprot ID
P00451
Uniprot Name
Coagulation factor VIII
Molecular Weight
267007.42 Da
References
  1. Brown R: Recombinant von Willebrand factor for severe gastrointestinal bleeding unresponsive to other treatments in a patient with type 2A von Willebrand disease: a case report. Blood Coagul Fibrinolysis. 2017 Oct;28(7):570-575. doi: 10.1097/MBC.0000000000000632. [Article]
  2. Gill JC, Castaman G, Windyga J, Kouides P, Ragni M, Leebeek FW, Obermann-Slupetzky O, Chapman M, Fritsch S, Pavlova BG, Presch I, Ewenstein B: Hemostatic efficacy, safety, and pharmacokinetics of a recombinant von Willebrand factor in severe von Willebrand disease. Blood. 2015 Oct 22;126(17):2038-46. doi: 10.1182/blood-2015-02-629873. Epub 2015 Aug 3. [Article]
  3. FDA Approved Drug Products: VONVENDI [von Willebrand factor (recombinant)] lyophilized powder for solution, for intravenous injection [Link]
Kind
Protein
Organism
Humans
Pharmacological action
Yes
Actions
Binder
General Function
Platelet-derived growth factor binding
Specific Function
Type I collagen is a member of group I collagen (fibrillar forming collagen).
Gene Name
COL1A1
Uniprot ID
P02452
Uniprot Name
Collagen alpha-1(I) chain
Molecular Weight
138941.105 Da
References
  1. Brown R: Recombinant von Willebrand factor for severe gastrointestinal bleeding unresponsive to other treatments in a patient with type 2A von Willebrand disease: a case report. Blood Coagul Fibrinolysis. 2017 Oct;28(7):570-575. doi: 10.1097/MBC.0000000000000632. [Article]
  2. Gill JC, Castaman G, Windyga J, Kouides P, Ragni M, Leebeek FW, Obermann-Slupetzky O, Chapman M, Fritsch S, Pavlova BG, Presch I, Ewenstein B: Hemostatic efficacy, safety, and pharmacokinetics of a recombinant von Willebrand factor in severe von Willebrand disease. Blood. 2015 Oct 22;126(17):2038-46. doi: 10.1182/blood-2015-02-629873. Epub 2015 Aug 3. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Unknown
Actions
Ligand
General Function
Mediates the endocytosis of plasma glycoproteins to which the terminal sialic acid residue on their complex carbohydrate moieties has been removed. The receptor recognizes terminal galactose and N-acetylgalactosamine units. After ligand binding to the receptor, the resulting complex is internalized and transported to a sorting organelle, where receptor and ligand are disassociated. The receptor then returns to the cell membrane surface.
Specific Function
Asialoglycoprotein receptor activity
Gene Name
ASGR1
Uniprot ID
P07306
Uniprot Name
Asialoglycoprotein receptor 1
Molecular Weight
33185.86 Da
References
  1. Lenting PJ, Christophe OD, Denis CV: von Willebrand factor biosynthesis, secretion, and clearance: connecting the far ends. Blood. 2015 Mar 26;125(13):2019-28. doi: 10.1182/blood-2014-06-528406. Epub 2015 Feb 23. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Unknown
General Function
Receptor activity
Specific Function
Endocytic receptor involved in endocytosis and in phagocytosis of apoptotic cells. Required for early embryonic development. Involved in cellular lipid homeostasis. Involved in the plasma clearance...
Gene Name
LRP1
Uniprot ID
Q07954
Uniprot Name
Prolow-density lipoprotein receptor-related protein 1
Molecular Weight
504601.695 Da
References
  1. Lenting PJ, Christophe OD, Denis CV: von Willebrand factor biosynthesis, secretion, and clearance: connecting the far ends. Blood. 2015 Mar 26;125(13):2019-28. doi: 10.1182/blood-2014-06-528406. Epub 2015 Feb 23. [Article]

Enzymes

Kind
Protein
Organism
Humans
Pharmacological action
No
Actions
Substrate
General Function
Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation.
Specific Function
Calcium ion binding
Gene Name
ADAMTS13
Uniprot ID
Q76LX8
Uniprot Name
A disintegrin and metalloproteinase with thrombospondin motifs 13
Molecular Weight
153603.05 Da
References
  1. Chung MC, Popova TG, Jorgensen SC, Dong L, Chandhoke V, Bailey CL, Popov SG: Degradation of circulating von Willebrand factor and its regulator ADAMTS13 implicates secreted Bacillus anthracis metalloproteases in anthrax consumptive coagulopathy. J Biol Chem. 2008 Apr 11;283(15):9531-42. doi: 10.1074/jbc.M705871200. Epub 2008 Feb 8. [Article]

Drug created at October 21, 2016 03:34 / Updated at February 03, 2022 06:26