Von Willebrand Factor And The Mechanisms Of Platelet Function (Biotechnology Intelligence Unit)

by Z.M Ruggeri

Publisher: Springer

Written in English
Cover of: Von Willebrand Factor And The Mechanisms Of Platelet Function (Biotechnology Intelligence Unit) | Z.M Ruggeri
Published: Pages: 257 Downloads: 33
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Subjects:

  • Cellular biology,
  • Haematology,
  • Immunology,
  • Science,
  • Medical / Nursing,
  • Science/Mathematics,
  • Hematology,
  • Life Sciences - Biology - General,
  • Physiology,
  • Life Sciences - Cytology,
  • Aggregation,
  • Blood platelets,
  • Pathophysiology,
  • Physiological effect,
  • Von Willebrand factor
  • The Physical Object
    FormatHardcover
    Number of Pages257
    ID Numbers
    Open LibraryOL12778071M
    ISBN 103540647090
    ISBN 109783540647096

  Heparin-induced thrombocytopenia (HIT) is a prothrombotic disorder mediated by complexes between platelet factor 4 (PF4) and heparin or other polyanions, but the risk of thrombosis extends beyond exposure to heparin implicating other PF4 partners. Abstract: von Willebrand disease is a common inherited bleeding disorder characterized by excessive mucocutaneous bleeding. Characteristic bleeding symptoms include epistaxis, easy bruising, oral. Tests for primary hemostasis involve evaluation of platelet number and function, and assessment of von Willebrand factor. Identification of blood vessel wall problems require the appropriate clinical signs (e.g. skin hyperextensibility with fragility in cats with Ehlers-Danlos syndrome) and histopathologic evaluation of biopsies. The ristocetin-induced platelet aggregation (RIPA) is an ex vivo assay for live platelet measures platelet aggregation with the help of von Willebrand factor (vWF) and exogenous antibiotic ristocetin added in a graded fashion. It is similar to the ristocetin cofactor assay but has the added benefit in that it helps in the diagnosis of type 2B/pseudo von Willebrand disease (vWD) and.

Von Willebrand disease type 2 is characterized by qualitative deficiency and functional anomalies of von Willebrand factor. It is divided in different subtypes including 2A, 2B, 2M and 2N (Normandy variant). The mutant VWF protein in types 2A, 2B and 2M are defective in their platelet-dependent function, whereas the mutant protein in type 2N is. This identifies “primary haemostasis” as the interaction between platelets, von Willebrand factor (vWF) and subendothelial collagen that results in the formation of a platelet plug. This plug provides a surface for the assembly of coagulation proteins required for fibrin formation, known as “secondary haemostasis” (McMichael , Wei. Erik Adolf von Willebrand (1 February – 12 September ) was a Finnish physician who made major contributions to hematology. Von Willebrand disease and von Willebrand factor are named after him. He also researched metabolism, obesity and gout, and was one of the first Finnish physicians to use insulin to treat a diabetic coma.. Von Willebrand qualified in medicine in from the. Start studying Hematology chapter Learn vocabulary, terms, and more with flashcards, games, and other study tools. Search. von Willebrand factor B) Christmas factor C) Tissue factor D) Stuart-Prower factor Platelet factor III facilitates _____ formation by the intrinsic coagulation system. A) Plasmin B) Thrombin.

Coagulation, also known as clotting, is the process by which blood changes from a liquid to a gel, forming a blood potentially results in hemostasis, the cessation of blood loss from a damaged vessel, followed by mechanism of coagulation involves activation, adhesion and aggregation of platelets, as well as deposition and maturation of fibrin. Mechanism of modulation of von Willebrand factor by ristocetin and botrocetin. Biochemistry. ;– Dong JF, Berndt MC, Schade A, McIntire LV, Andrews RK, López JA. Disorders of Hemostasis study guide by Patch includes 70 questions covering vocabulary, terms and more. Circulates in the Plasma bound to von Willebrand Factor, which stabilizes it, preventing it from destruction and regulating its synthesis Normal Platelet Function This narrows it down to Factor .   Von Willebrand Disease (vWd) The most common canine hereditary bleeding disorder. Three subtype classifications which are dependent on the severity of clinical signs, mode of inheritance, and biochemical abnormalities of von Willebrand factor protein (vWf). Type 1 vWd has been observed in many canine breeds and in cats.

Von Willebrand Factor And The Mechanisms Of Platelet Function (Biotechnology Intelligence Unit) by Z.M Ruggeri Download PDF EPUB FB2

Von Willebrand factor and the mechanisms of platelet function. [Zaverio M Ruggeri;] -- "This book deals with hemostasis, the mechanism that controls thrombosis and bleeding after tissue trauma.

In spite of the fact that thrombotic vascular diseases are the most common cause of. Hemostasis and pathological thrombus formation are dynamic processes that require multiple adhesive receptor-ligand interactions, with blood platelets at the heart of such events.

Many studies have contributed to shed light on the importance of von Willebrand factor (VWF) interaction with its platelet receptors, glycoprotein (GP) Ib-IX-V and αIIbβ3 integrin, in promoting primary platelet Cited by:   Many studies have contributed to shed light on the importance of von Willebrand factor (VWF) interaction with its platelet receptors, glycoprotein (GP) Ib-IX-V and αIIbβ3 integrin, in promoting primary platelet adhesion and aggregation following vessel by: Many studies have contributed to shed light on the importance of von Willebrand factor (VWF) interaction with its platelet receptors, glycoprotein (GP) Ib-IX-V and αIIbβ3 integrin, in promoting.

Von Willebrand disease (VWD) is the most common congenital clotting factor disorder. Von Willebrand disease is caused by a qualitative or quantitative defect in VWF.

Von Willebrand factor is a multimeric glyco-protein that binds collagen at the site of vascular injury and is involved in platelet File Size: KB. The hypothesis that von Willebrand factor (vWF) binding to platelet membrane glycoprotein Ib (GpIb) initiates intracellular pathways of platelet activation was studied.

Platelet biology and functions: new concepts and clinical perspectives thrombus formation poststenosis in a von Willebrand factor-dependent manner.

and functions: new concepts and. Von Willebrand factor (VWF) is a large multimeric glycoprotein produced in endothelial cells and megakaryocytes and present in subendothelial matrix, blood plasma and platelets.

VWF mediates adhesion and aggregation of platelets at sites of vascular injury, processes that are critical for both haemostasis and thrombosis.

collagen, ibrutinib, platelet function, von willebrand disease, platelet aggregation Introduction The Bruton tyrosine kinase (Btk) is an essential actor downstream of the B-cell receptor, and its covalent inhibitor, ibrutinib, has recently been approved for therapies of relapsed chronic lymphocytic leukemia (CLL) and mantle cell lymphoma (MCL.

Three sisters had died before the age of four, one living sister, aged three, also was severely affected. von Willebrand had thought that it was a disorder of platelet function or a vascular defect as a possible cause of the by: Von Willebrand factor (vWF, or VWF), glycoprotein that plays an important role in stopping the escape of blood from vessels (hemostasis) following vascular injury.

Von Willebrand factor (VWF) works by mediating the adherence of platelets to one another and to sites of vascular damage. VWF binds to a protein complex made up of the glycoproteins Ib, IX, and V on the surfaces of platelets. platelet function disorders.

Further references not initially identified in the search but referenced within these articles were also reviewed. Review articles and textbooks were used to inform our discussion of the physiology of platelets, and current understanding of the biochemical mechanisms involved in platelet activation.

6 Von Willebrand factor and platelet function. The second important function of vWF is to be a carrier forprotecting it from degradation and playing a role in its activation by thrombin.

The mechanisms that control the synthesis of vWF should be an exciting area of further research. The von Willebrand factor (vWF) promotes platelet interaction with the damaged vessel wall under conditions of shear stress.

Two platelet receptors have been shown to bind vWF, glycoprotein (GP) Ib and GP IIb/ binding of vWF to GP Ib leads to the initial contact of platelets with the subendothelium (i.e., platelet adhesion) 2, 3, the process by which the platelets attach to the. Von Willebrand Factor's primary function is binding to other proteins, in particular factor VIII, and it is important in platelet adhesion to wound sites.

It is not an enzyme and, thus, has no catalytic activity. The diagnosis is based on von Willebrand factor antigen, von Willebrand factor activity assay, FVIII coagulant activity and some other additional tests.

Results should be analyzed within the context of blood group. von Willebrand factor multimer analysis is essential for typing and sub typing the disease. Von Willebrand factor (VWF) is a large multimeric glycoprotein that performs two critical functions in primary hemostasis: it acts as a bridging molecule at sites of vascular injury for normal platelet adhesion, and under high shear conditions, it promotes platelet aggregation.

Plasma von Willebrand factor (VWF) has been identified as an indispensable factor for platelet adhesion and thrombus formation on a collagen surface under flow conditions.

VWF binds to collagen and then tethers platelets to the collagen surface through interaction with platelet glycoprotein Ib and also contributes to the thrombus formation on.

The aggregation response depends upon the most abundant platelet membrane protein, integrin αIIbβ3 (also known as glycoprotein [GP] IIb-IIIa), and its ability to serve as a receptor for extracellular ligands. Occupancy of αIIbβ3 by fibrinogen or von Willebrand factor can.

The development of acquired von Willebrand syndrome (AVWS) has been identified as relevant pathology during ECMO. This study was performed to determine the onset of AVWS after implantation and the recovery of von Willebrand factor (VWF) parameters after explantation of ECMO in a large cohort of patients.

The adhesive protein von Willebrand factor (VWF) contributes to platelet function by mediating the initiation and progression of thrombus formation at sites of vascular injury.

In recent years there has been considerable progress in explaining the biological properties of VWF, including the structural and functional characteristics of specific domains.

Introduction. Mature von Willebrand factor (VWF) is a multimeric protein composed of a variable number of identical subunits, each consisting of amino acid residues and up to 22 carbohydrate side ts are disulphide-bonded into dimers of approximately kDa, which in turn are disulphide-linked into multimers of increasing size that can reach a molecular mass as high as 20 MDa.

When this factor binds to collagen, thrombocytes can adhere to a distinct part of the von Willebrand molecule with its GP Ib/V/IX-complex and thereby accumulate at the site of damage.

A defect or malfunction in these GP Ib/V/IX-complexes is linked to a bleeding complication: The. Among the many putative mechanisms for platelet-endothelial interactions, increased endothelial-associated von Willebrand factor, particularly in a multimerized form, which interacts with platelet glycoproteins and integrins, is a major factor and represents a.

Von Willebrand disease (VWD) is caused by mutations that lead to an impairment in the synthesis or function of von Willebrand factor (VWF). There are also acquired forms of VWD that are caused by several different pathophysiologic mechanisms.

The classification and pathophysiology of. Von Willebrand disease (vWD) is the most common hereditary blood-clotting disorder in humans. An acquired form can sometimes result from other medical conditions. It arises from a deficiency in the quality or quantity of von Willebrand factor (vWF), a multimeric protein that is required for platelet well as humans, it is known to affect several breeds of dogs.

Ruggeri, Z.M. Structure of von Willebrand factor and its function in platelet adhesion and thrombus formation. Balli re's Clinical Hematology, in press, Ruggeri, Z.M.

Platelet. Zeigler ZR, Megaludis A, Fraley DS. Desmopressin (d-DAVP) effects on platelet rheology and von Willebrand factor activities in uremia.

Am J Hematol ; Gordz S, Mrowietz C, Pindur G, et al. Effect of desmopressin (DDAVP) on platelet membrane glycoprotein expression in patients with von Willebrand's disease. The first video in a series of three on the topic of clotting (haemostasis).

In this video we discuss how platelets work. We also look at the drugs used in medicine to inhibit the action of.

INTRODUCTION. Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Diagnosis can be challenging; some individuals with low von Willebrand factor (VWF) levels may not actually have VWD (or any bleeding disorder), whereas others who have never had a bleeding challenge or never been tested have a significant bleeding risk from VWD that would benefit from evaluation.

Further research is also needed to better understand the mechanism of how the ABO blood group on platelets alters binding to VWF. References: Dunne E, Qin MQ, Shaqfeh ES, O’Sullivan JM, et al. Blood group alters platelet binding kinetics to von Willebrand factor and consequently platelet function.

Blood ; (12); form of endothelial cell von Willebrand factor is released by the regulated pathway. British Journal of Haematology. ; 79 (2) Endothelial Dysfunction - Old Concepts and New Challenges Introduction. The path of the adhesive glycoprotein von Willebrand factor (VWF) has been traced since when a 5‐year‐old girl from an island off the coast of Finland was brought to Dr Erik von Willebrand for evaluation of a severe bleeding disorder [].The cause of the bleeding was first thought to be a platelet defect, but in the following decades it was pinpointed to be a failure in.