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Article ; Online: Glanzmann Thrombasthenia 10 Years Later: Progress Made and Future Directions.

Nurden, Alan T / Nurden, Paquita

2024

Abstract: Glanzmann thrombasthenia (GT) is the most common inherited platelet disorder (IPD) with mucocutaneous bleeding and a failure of platelets to aggregate when stimulated. The molecular cause is insufficient or defective αIIbβ3, an integrin encoded by ... ...

Abstract	Glanzmann thrombasthenia (GT) is the most common inherited platelet disorder (IPD) with mucocutaneous bleeding and a failure of platelets to aggregate when stimulated. The molecular cause is insufficient or defective αIIbβ3, an integrin encoded by the
Language	English
Publishing date	2024-03-18
Publishing country	United States
Document type	Journal Article
ZDB-ID	196901-8
ISSN	1098-9064 ; 0094-6176
ISSN (online)	1098-9064
ISSN	0094-6176
DOI	10.1055/s-0044-1782519
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Article ; Online: The GPIIb-IIIa defect of platelets in Glanzmann thrombasthenia.

Nurden, Alan T

Haematologica

2023 Volume 108, Issue 4, Page(s) 937–938

MeSH term(s)	Humans ; Thrombasthenia/genetics ; Blood Platelets ; Platelet Glycoprotein GPIIb-IIIa Complex ; Platelet Aggregation
Chemical Substances	Platelet Glycoprotein GPIIb-IIIa Complex
Language	English
Publishing date	2023-04-01
Publishing country	Italy
Document type	Journal Article
ZDB-ID	2333-4
ISSN	1592-8721 ; 0017-6567 ; 0390-6078
ISSN (online)	1592-8721
ISSN	0017-6567 ; 0390-6078
DOI	10.3324/haematol.2023.282836
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Article: Glanzmann Thrombasthenia 10 Years Later: Progress Made and Future Directions

Nurden, Alan T. / Nurden, Paquita

Seminars in Thrombosis and Hemostasis

(Precision Medicine in Rare Bleeding Disorders)

2024

Series title	Precision Medicine in Rare Bleeding Disorders
Abstract	Glanzmann thrombasthenia (GT) is the most common inherited platelet disorder (IPD) with mucocutaneous bleeding and a failure of platelets to aggregate when stimulated. The molecular cause is insufficient or defective αIIbβ3, an integrin encoded by the ITGA2B and ITGB3 genes. On activation αIIbβ3 undergoes conformational changes and binds fibrinogen (Fg) and other proteins to join platelets in the aggregate. The application of next-generation sequencing (NGS) to patients with IPDs has accelerated genotyping for GT; progress accompanied by improved mutation curation. The evaluation by NGS of variants in other hemostasis and vascular genes is a major step toward understanding why bleeding varies so much between patients. The recently discovered role for glycoprotein VI in thrombus formation, through its binding to fibrin and surface-bound Fg, may offer a mechanosensitive back-up for αIIbβ3, especially at sites of inflammation. The setting up of national networks for IPDs and GT is improving patient care. Hematopoietic stem cell therapy provides a long-term cure for severe cases; however, prophylaxis by monoclonal antibodies designed to accelerate fibrin formation at injured sites in the vasculature is a promising development. Gene therapy using lentil-virus vectors remains a future option with CRISPR/Cas9 technologies offering a promising alternative route.
Keywords	Glanzmann thrombasthenia ; αIIbβ3 integrin ; biology ; mutations ; prophylaxis ; gene therapy
Language	English
Publishing date	2024-03-18
Publisher	Thieme Medical Publishers, Inc.
Publishing place	Stuttgart ; New York
Document type	Article
ZDB-ID	196901-8
ISSN	1098-9064 ; 0094-6176
ISSN (online)	1098-9064
ISSN	0094-6176
DOI	10.1055/s-0044-1782519
Database	Thieme publisher's database

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Article ; Online: Molecular basis of clot retraction and its role in wound healing.

Nurden, Alan T

Thrombosis research

2022 Volume 231, Page(s) 159–169

Abstract: Clot retraction is important for the prevention of bleeding, in the manifestations of thrombosis and for tissue repair. The molecular mechanisms behind clot formation are complex. Platelet involvement begins with adhesion at sites of vessel injury ... ...

Abstract	Clot retraction is important for the prevention of bleeding, in the manifestations of thrombosis and for tissue repair. The molecular mechanisms behind clot formation are complex. Platelet involvement begins with adhesion at sites of vessel injury followed by platelet aggregation, thrombin generation and fibrin production. Other blood cells incorporate into a fibrin mesh that is consolidated by FXIIIa-mediated crosslinking and platelet contractile activity. The latter results in the asymmetric redistribution of erythrocytes into a tighter central mass providing the clot with stability and resistance to fibrinolysis. Integrin αIIbβ3 on platelets is the key player in these events, bridging fibrin and the platelet cytoskeleton. Glycoprotein VI participates in thrombus formation but not in the retraction. Rheological and environmental factors influence clot construction with retraction driven by the platelet cytoskeleton with actomyosin acting as the motor. Activated platelets provide procoagulant activity stimulating thrombin generation together with the release of a plethora of biologically active proteins and substances from storage pools; many form chemotactic gradients within the fibrin or the underlying matrix. Also released are newly synthesized metabolites and lipid-rich vesicles that circulate within the vasculature and mimic platelet functions. Platelets and their released elements play key roles in wound healing. This includes promoting stem cell and mesenchymal stromal cell recruitment, fibroblast and endothelial cell migration, angiogenesis and matrix formation. These properties have led to the use of autologous clots in therapies designed to accelerate tissue repair while offering the potential for genetic manipulation in both inherited and acquired diseases.
MeSH term(s)	Humans ; Clot Retraction ; Thrombin/metabolism ; Blood Platelets/metabolism ; Wound Healing ; Thrombosis ; Fibrin/metabolism
Chemical Substances	Thrombin (EC 3.4.21.5) ; Fibrin (9001-31-4)
Language	English
Publishing date	2022-08-19
Publishing country	United States
Document type	Journal Article
ZDB-ID	121852-9
ISSN	1879-2472 ; 0049-3848
ISSN (online)	1879-2472
ISSN	0049-3848
DOI	10.1016/j.thromres.2022.08.010
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Zs.A 863: Show issues

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Article ; Online: Inherited thrombocytopenias: history, advances and perspectives.

Nurden, Alan T / Nurden, Paquita

Haematologica

2020 Volume 105, Issue 8, Page(s) 2004–2019

Abstract: Over the last 100 years the role of platelets in hemostatic events and their production by megakaryocytes have gradually been defined. Progressively, thrombocytopenia was recognized as a cause of bleeding, first through an acquired immune disorder; then, ...

Abstract	Over the last 100 years the role of platelets in hemostatic events and their production by megakaryocytes have gradually been defined. Progressively, thrombocytopenia was recognized as a cause of bleeding, first through an acquired immune disorder; then, since 1948, when Bernard-Soulier syndrome was first described, inherited thrombocytopenia became a fascinating example of Mendelian disease. The platelet count is often severely decreased and platelet size variable; associated platelet function defects frequently aggravate bleeding. Macrothrombocytopenia with variable proportions of enlarged platelets is common. The number of circulating platelets will depend on platelet production, consumption and lifespan. The bulk of macrothrombocytopenias arise from defects in megakaryopoiesis with causal variants in transcription factor genes giving rise to altered stem cell differentiation and changes in early megakaryocyte development and maturation. Genes encoding surface receptors, cytoskeletal and signaling proteins also feature prominently and Sanger sequencing associated with careful phenotyping has allowed their early classification. It quickly became apparent that many inherited thrombocytopenias are syndromic while others are linked to an increased risk of hematologic malignancies. In the last decade, the application of next-generation sequencing, including whole exome sequencing, and the use of gene platforms for rapid testing have greatly accelerated the discovery of causal genes and extended the list of variants in more common disorders. Genes linked to an increased platelet turnover and apoptosis have also been identified. The current challenges are now to use next-generation sequencing in first-step screening and to define bleeding risk and treatment better.
MeSH term(s)	Bernard-Soulier Syndrome ; Blood Platelets ; Humans ; Megakaryocytes ; Thrombocytopenia/diagnosis ; Thrombocytopenia/genetics ; Thrombopoiesis/genetics
Language	English
Publishing date	2020-06-11
Publishing country	Italy
Document type	Journal Article ; Review
ZDB-ID	2333-4
ISSN	1592-8721 ; 0017-6567 ; 0390-6078
ISSN (online)	1592-8721
ISSN	0017-6567 ; 0390-6078
DOI	10.3324/haematol.2019.233197
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Article ; Online: Acquired Glanzmann thrombasthenia: From antibodies to anti-platelet drugs.

Nurden, Alan T

Blood reviews

2019 Volume 36, Page(s) 10–22

Abstract: In contrast to the inherited platelet disorder given by mutations in the ITGA2B and ITGB3 genes, mucocutaneous bleeding from a spontaneous inhibition of normally expressed αIIbβ3 characterizes acquired Glanzmann thrombasthenia (GT). Classically, it is ... ...

Abstract	In contrast to the inherited platelet disorder given by mutations in the ITGA2B and ITGB3 genes, mucocutaneous bleeding from a spontaneous inhibition of normally expressed αIIbβ3 characterizes acquired Glanzmann thrombasthenia (GT). Classically, it is associated with autoantibodies or paraproteins that block platelet aggregation without causing a fall in platelet count. However, inhibitory antibodies to αIIbβ3 are widely associated with primary immune thrombocytopenia (ITP), occur in secondary ITP associated with leukemia and related disorders, solid cancers and myeloma, other autoimmune diseases, following organ transplantation while cytoplasmic dysregulation of αIIbβ3 function features in myeloproliferative and myelodysplastic syndromes. Antibodies to αIIbβ3 occur during viral and bacterial infections, while drug-dependent antibodies reacting with αIIbβ3 are a special case. Direct induction of acquired GT is a feature of therapies that block platelets in coronary artery disease. This review looks at these conditions, emphasizing molecular mechanisms, therapy, patient management and future directions for research.
MeSH term(s)	Antibodies/pharmacology ; Antibodies/therapeutic use ; Dual Anti-Platelet Therapy/methods ; Humans ; Thrombasthenia/drug therapy ; Thrombasthenia/genetics ; Thrombasthenia/pathology
Chemical Substances	Antibodies
Language	English
Publishing date	2019-03-20
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	639015-8
ISSN	1532-1681 ; 0268-960X
ISSN (online)	1532-1681
ISSN	0268-960X
DOI	10.1016/j.blre.2019.03.004
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Zs.A 2207: Show issues

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Article ; Online: Clinical significance of altered collagen-receptor functioning in platelets with emphasis on glycoprotein VI.

Nurden, Alan T

Blood reviews

2019 Volume 38, Page(s) 100592

Abstract: Much interest surrounds the receptors α2β1 and glycoprotein VI (GPVI) whose synchronized action mediates the attachment and activation of platelets on collagen, essential for preventing blood loss but also the most thrombogenic component of the vessel ... ...

Abstract	Much interest surrounds the receptors α2β1 and glycoprotein VI (GPVI) whose synchronized action mediates the attachment and activation of platelets on collagen, essential for preventing blood loss but also the most thrombogenic component of the vessel wall. Subject to density variations on platelets through natural polymorphisms, the absence of α2β1 or GPVI uniquely leads to a substantial block of hemostasis without causing major bleeding. Specific to the megakaryocyte lineage, GPVI and its signaling pathways are most promising targets for anti-thrombotic therapy. This review looks at the clinical consequences of the loss of collagen receptor function with emphasis on both the inherited and acquired loss of GPVI with brief mention of mouse models when necessary. A detailed survey of rare case reports of patients with inherited disease-causing variants of the GP6 gene is followed by an assessment of the causes and clinical consequences of acquired GPVI deficiency, a more frequent finding most often due to antibody-induced platelet GPVI shedding. Release of soluble GPVI is brought about by platelet metalloproteinases; a process induced by ligand or antibody binding to GPVI or even high shear forces. Also included is an assessment of the clinical importance of GPVI-mediated platelet interactions with fibrin and of the promise shown by the pharmacological inhibition of GPVI in a cardiovascular context. The role for GPVI in platelet function in inflammation and in the evolution and treatment of major illnesses such as rheumatoid arthritis, cancer and sepsis is also discussed.
MeSH term(s)	Animals ; Blood Platelets/metabolism ; Collagen/metabolism ; Humans ; Integrin alpha2beta1/metabolism ; Platelet Activation ; Platelet Membrane Glycoproteins/metabolism ; Receptors, Collagen/metabolism ; Signal Transduction
Chemical Substances	Integrin alpha2beta1 ; Platelet Membrane Glycoproteins ; Receptors, Collagen ; platelet membrane glycoprotein VI ; Collagen (9007-34-5)
Language	English
Publishing date	2019-07-22
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	639015-8
ISSN	1532-1681 ; 0268-960X
ISSN (online)	1532-1681
ISSN	0268-960X
DOI	10.1016/j.blre.2019.100592
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Article ; Online: Is the mysterious platelet receptor GPV an unsuspected major target for platelet autoantibodies?

Nurden, Paquita / Nurden, Alan T

Haematologica

2019 Volume 104, Issue 6, Page(s) 1103–1105

MeSH term(s)	Autoantibodies ; Blood Platelets ; Humans ; Platelet Glycoprotein GPIb-IX Complex ; Purpura, Thrombocytopenic, Idiopathic ; Thrombocytopenia
Chemical Substances	Autoantibodies ; Platelet Glycoprotein GPIb-IX Complex
Language	English
Publishing date	2019-05-31
Publishing country	Italy
Document type	Editorial ; Comment
ZDB-ID	2333-4
ISSN	1592-8721 ; 0017-6567 ; 0390-6078
ISSN (online)	1592-8721
ISSN	0017-6567 ; 0390-6078
DOI	10.3324/haematol.2018.214908
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Article ; Online: Personal reflections on the early contributions of Gus Born to platelet research.

Nurden, Alan T

Platelets

2018 , Page(s) 1–5

Abstract: Professor GVR Born, Gus to his friends, was one of the great pioneers of platelet research. My early memories of him have enabled me to look back at his early years in Oxford and London. A brilliant and generous man with always the time to discuss and ... ...

Abstract	Professor GVR Born, Gus to his friends, was one of the great pioneers of platelet research. My early memories of him have enabled me to look back at his early years in Oxford and London. A brilliant and generous man with always the time to discuss and advise he was instrumental in deciphering the principle stages of the aggregation of blood platelets by ADP, a path aided by his development and use of the platelet aggregometer. He applied his knowledge to the real time analysis of platelet and leukocyte involvement in thrombus formation in animal models and to the development of atherosclerosis and thrombosis and their pharmacological inhibition. What follows is a personal account of the major steps in this early work and of the actors involved.
Language	English
Publishing date	2018-09-05
Publishing country	England
Document type	Journal Article
ZDB-ID	1034283-7
ISSN	1369-1635 ; 0953-7104
ISSN (online)	1369-1635
ISSN	0953-7104
DOI	10.1080/09537104.2018.1513477
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Article ; Online: The biology of the platelet with special reference to inflammation, wound healing and immunity.

Nurden, Alan T

Frontiers in bioscience (Landmark edition)

2018 Volume 23, Issue 4, Page(s) 726–751

Abstract: While platelets have long been known to be essential for maintaining hemostasis in the vasculature, their role in tissue repair, inflammation and innate and adaptive immunity is a more recent science. The ability of platelets to attach to the vessel wall, ...

Abstract	While platelets have long been known to be essential for maintaining hemostasis in the vasculature, their role in tissue repair, inflammation and innate and adaptive immunity is a more recent science. The ability of platelets to attach to the vessel wall, form aggregates and promote fibrin formation, key elements of blood clotting, has been said to both favor and dampen inflammation, to fight infection and to assure an adequate immune response. To fulfill their different roles platelets often synchronize with leukocytes and cells of the immune system. But just as the molecular pathways of platelets in preventing blood loss can lead to arterial thrombosis and stroke if occurring in an uncontrolled manner, the failure to control inflammation can lead to sepsis and inadequate platelet function and can aggravate many major illnesses. This review is aimed to present a global picture of multifaceted platelet biology and platelet involvement in selected non-hemostatic events.
MeSH term(s)	Adaptive Immunity/immunology ; Apoptosis/immunology ; Blood Platelets/immunology ; Hemostasis/immunology ; Humans ; Immunity, Innate/immunology ; Inflammation/immunology ; Sepsis/immunology ; Wound Healing/immunology
Language	English
Publishing date	2018-01-01
Publishing country	Singapore
Document type	Journal Article ; Review
ZDB-ID	2704569-9
ISSN	2768-6698 ; 1093-9946
ISSN (online)	2768-6698
ISSN	1093-9946
DOI	10.2741/4613
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