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Article: Interaction of von Willebrand factor with platelets and the vessel wall.

Ruggeri, Z M / Mendolicchio, G L

2015 Volume 35, Issue 3, Page(s) 211–224

Abstract: The initiation of thrombus formation at sites of vascular injury to secure haemostasis after tissue trauma requires the interaction of surface-exposed von Willebrand factor (VWF) with its primary platelet receptor, the glycoprotein (GP) Ib-IX-V complex. ... ...

Abstract	The initiation of thrombus formation at sites of vascular injury to secure haemostasis after tissue trauma requires the interaction of surface-exposed von Willebrand factor (VWF) with its primary platelet receptor, the glycoprotein (GP) Ib-IX-V complex. As an insoluble component of the extracellular matrix (ECM) of endothelial cells, VWF can directly initiate platelet adhesion. Circulating plasma VWF en-hances matrix VWF activity by binding to structures that become exposed to flowing blood, notably collagen type I and III in deeper layers of the vessel along with microfibrillar collagen type VI in the subendothelium. Moreover, plasma VWF is required to support platelet-to-platelet adhesion - i. e. aggregation - which promotes thrombus growth and consolidation. For these reasons, understanding how plasma VWF interaction with platelet receptors is regulated, particularly any distinctive features of GPIb binding to soluble as opposed to immobilized VWF, is of paramount importance in vascular biology. This brief review will highlight knowledge acquired and key problems that remain to be solved to elucidate fully the role of VWF in normal haemostasis and pathological thrombosis.
MeSH term(s)	Animals ; Blood Platelets/immunology ; Blood Platelets/pathology ; Blood Vessels/immunology ; Blood Vessels/pathology ; Hemostasis/immunology ; Humans ; Models, Cardiovascular ; Models, Immunological ; Platelet Activation/immunology ; Platelet Adhesiveness/immunology ; Thrombosis/immunology ; Thrombosis/pathology ; Vascular System Injuries/immunology ; Vascular System Injuries/pathology
Language	English
Publishing date	2015
Publishing country	Germany
Document type	Journal Article ; Review
ZDB-ID	801512-0
ISSN	0720-9355
ISSN	0720-9355
DOI	10.5482/HAMO-14-12-0081
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Halide Remixing under Device Operation Imparts Stability on Mixed-Cation Mixed-Halide Perovskite Solar Cells.

Ruggeri, Edoardo / Anaya, Miguel / Gałkowski, Krzysztof / Abfalterer, Anna / Chiang, Yu-Hsien / Ji, Kangyu / Andaji-Garmaroudi, Zahra / Stranks, Samuel D

Advanced materials (Deerfield Beach, Fla.)

2022 Volume 34, Issue 36, Page(s) e2202163

Abstract: Mixed-halide mixed-cation hybrid perovskites are among the most promising perovskite compositions for application in a variety of optoelectronic devices due to their high performance, low cost, and bandgap-tuning capabilities. Instability pathways such ... ...

Abstract	Mixed-halide mixed-cation hybrid perovskites are among the most promising perovskite compositions for application in a variety of optoelectronic devices due to their high performance, low cost, and bandgap-tuning capabilities. Instability pathways such as those driven by ionic migration, however, continue to hinder their further progress. Here, an operando variable-pitch synchrotron grazing-incidence wide-angle X-ray scattering technique is used to track the surface and bulk structural changes in mixed-halide mixed-cation perovskite solar cells under continuous load and illumination. By monitoring the evolution of the material structure, it is demonstrated that halide remixing along the electric field and illumination direction during operation hinders phase segregation and limits device instability. Correlating the evolution with directionality- and depth-dependent analyses, it is proposed that this halide remixing is induced by an electrostrictive effect acting along the substrate out-of-plane direction. However, this stabilizing effect is overwhelmed by competing halide demixing processes in devices exposed to humid air or with poorer starting performance. The findings shed new light on understanding halide de- and re-mixing competitions and their impact on device longevity. These operando techniques allow real-time tracking of the structural evolution in full optoelectronic devices and unveil otherwise inaccessible insights into rapid structural evolution under external stress conditions.
Language	English
Publishing date	2022-08-07
Publishing country	Germany
Document type	Journal Article
ZDB-ID	1474949-X
ISSN	1521-4095 ; 0935-9648
ISSN (online)	1521-4095
ISSN	0935-9648
DOI	10.1002/adma.202202163
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Article ; Online: Hematopoietic cell transplantation and cellular therapies in Europe 2022. CAR-T activity continues to grow; transplant activity has slowed: a report from the EBMT.

Passweg, Jakob R / Baldomero, Helen / Ciceri, Fabio / de la Cámara, Rafael / Glass, Bertram / Greco, Raffaella / Hazenberg, Mette D / Kalwak, Krzysztof / McLornan, Donal P / Neven, Bénédicte / Perić, Zinaida / Risitano, Antonio M / Ruggeri, Annalisa / Snowden, John A / Sureda, Anna

Bone marrow transplantation

2024

Abstract: In 2022, 46,143 HCT (19,011 (41.2%) allogeneic and 27,132 (58.8%) autologous) in 41,854 patients were reported by 689 European centers. 4329 patients received advanced cellular therapies, 3205 of which were CAR-T. An additional 2854 patients received DLI. ...

Abstract	In 2022, 46,143 HCT (19,011 (41.2%) allogeneic and 27,132 (58.8%) autologous) in 41,854 patients were reported by 689 European centers. 4329 patients received advanced cellular therapies, 3205 of which were CAR-T. An additional 2854 patients received DLI. Changes compared to the previous year were an increase in CAR-T treatments (+27%) and decrease in allogeneic (-4.0%) and autologous HCT (-1.7%). Main indications for allogeneic HCT were myeloid malignancies (10,433; 58.4%), lymphoid malignancies (4,674; 26.2%) and non-malignant disorders (2572; 14.4%). Main indications for autologous HCT were lymphomas (7897; 32.9%), PCD (13,694; 57.1%) and solid tumors (1593; 6.6%). In allogeneic HCT, use of sibling donors decreased by -7.7%, haploidentical donors by -6.3% and unrelated donors by -0.9%. Overall cord blood HCT decreased by -16.0%. Use of allogeneic, and to a lesser degree autologous HCT, decreased for lymphoid malignancies likely reflecting availability of new treatment modalities, including small molecules, bispecific antibodies, and CAR-T cells. Pediatric HCT activity remains stable (+0.3%) with differences between allogeneic and autologous HCT. Use of CAR-T continues to increase and reached a cumulative total of 9039 patients treated with wide differences across European countries. After many years of continuous growth, increase in application of HCT seems to have slowed down.
Language	English
Publishing date	2024-03-04
Publishing country	England
Document type	Journal Article
ZDB-ID	632854-4
ISSN	1476-5365 ; 0268-3369 ; 0951-3078
ISSN (online)	1476-5365
ISSN	0268-3369 ; 0951-3078
DOI	10.1038/s41409-024-02248-9
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Article: Estimation of the full shape of the crystalline lens

Martínez-Enríquez, Eduardo / Curatolo, Andrea / de Castro, Alberto / Birkenfeld, Judith S / González, Ana M / Mohamed, Ashik / Ruggeri, Marco / Manns, Fabrice / Fernando, Zvietcovich / Marcos, Susana

Biomedical optics express

2023 Volume 14, Issue 2, Page(s) 608–626

Abstract: Quantifying the full 3-D shape of the human crystalline lens is important for improving intraocular lens power or sizing calculations in treatments of cataract and presbyopia. In a previous work we described a novel method for the representation of the ... ...

Abstract	Quantifying the full 3-D shape of the human crystalline lens is important for improving intraocular lens power or sizing calculations in treatments of cataract and presbyopia. In a previous work we described a novel method for the representation of the full shape of the
Language	English
Publishing date	2023-01-05
Publishing country	United States
Document type	Journal Article
ZDB-ID	2572216-5
ISSN	2156-7085
ISSN	2156-7085
DOI	10.1364/BOE.477557
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Article: Interaction of von Willebrand factor with platelets and the vessel wall

Ruggeri, Z. M. / Mendolicchio, G. L.

Hämostaseologie

2015 Volume 35, Issue 03, Page(s) 211–224

Abstract	The initiation of thrombus formation at sites of vascular injury to secure haemostasis after tissue trauma requires the interaction of surface-exposed von Willebrand factor (VWF) with its primary platelet receptor, the glycoprotein (GP) Ib-IX-V complex. As an insoluble component of the extracellular matrix (ECM) of endothelial cells, VWF can directly initiate platelet adhesion. Circulating plasma VWF en-hances matrix VWF activity by binding to structures that become exposed to flowing blood, notably collagen type I and III in deeper layers of the vessel along with microfibrillar collagen type VI in the sub endothelium. Moreover, plasma VWF is required to support platelet-to-platelet adhesion – i. e. aggregation – which promotes thrombus growth and consolidation. For these reasons, understanding how plasma VWF interaction with platelet receptors is regulated, particularly any distinctive features of GPIb binding to soluble as opposed to immobilized VWF, is of paramount importance in vascular biology. This brief review will highlight knowledge acquired and key problems that remain to be solved to elucidate fully the role of VWF in normal haemostasis and pathological thrombosis. Zu Beginn der Thrombusbildung in verletzten Gefäßabschnitten muss zur Sicherung der Hämostase nach einem Gewebetrauma der oberflächenexponierte von-Willebrand-Faktor (VWF) mit seinem primären Thrombozytenrezeptor, dem Glykoprotein(GP)-Ib-IXV-Komplex, interagieren. Als unlöslicher Bestandteil der extrazellulären Matrix (EZM) der Endothelzellen kann VWF die Thrombozytenadhäsion direkt auslösen. Im Plasma zirkulierender VWF erhöht die Aktivität des Matrix-VWF durch Bindung an Strukturen, die dem Blutstrom ausgesetzt werden, vor allem Kollagen I und III in tieferen Gefäßwandschichten sowie subendotheliales Kollagen VI der Mikrofibrillen. Außerdem wird der Plasma-VWF für die Adhäsion der Thrombozyten untereinander – d. h. die Aggregation – benötigt, durch die Wachstum und Konsolidierung des Thrombus gefördert werden. Deshalb ist es in der Gefäßbiologie wichtig zu verstehen, wie die Wechselwirkung zwischen Plasma-VWF und Plättchenrezeptoren reguliert wird, besonders alle Merkmale der Bindung von GPIb an löslichen oder gebundenen VWF. Diese kurze Übersicht beleuchtet die Erkenntnisse und Probleme, die zu lösen sind, um die Rolle des VWF bei der normalen Hämostase und bei pathologischen Thrombosen gänzlich aufzuklären.
Keywords	Von Willebrand factor ; Von-Willebrand-Faktor
Language	English
Publishing date	2015-03-01
Publisher	Schattauer GmbH
Publishing place	Stuttgart ; New York
Document type	Article
ZDB-ID	801512-0
ISSN	2567-5761 ; 0720-9355
ISSN (online)	2567-5761
ISSN	0720-9355
DOI	10.5482/HAMO-14-12-0081
Database	Thieme publisher's database

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Article ; Online: Enhanced surface nanoanalytics of transient biomolecular processes.

Miller, Alyssa / Chia, Sean / Toprakcioglu, Zenon / Hakala, Tuuli / Schmid, Roman / Feng, Yaduo / Kartanas, Tadas / Kamada, Ayaka / Vendruscolo, Michele / Ruggeri, Francesco Simone / Knowles, Tuomas P J

Science advances

2023 Volume 9, Issue 2, Page(s) eabq3151

Abstract: Fundamental knowledge of the physical and chemical properties of biomolecules is key to understanding molecular processes in health and disease. Bulk and single-molecule analytical methods provide rich information about biomolecules but often require ... ...

Abstract	Fundamental knowledge of the physical and chemical properties of biomolecules is key to understanding molecular processes in health and disease. Bulk and single-molecule analytical methods provide rich information about biomolecules but often require high concentrations and sample preparation away from physiologically relevant conditions. Here, we present the development and application of a lab-on-a-chip spray approach that combines rapid sample preparation, mixing, and deposition to integrate with a range of nanoanalytical methods in chemistry and biology, providing enhanced spectroscopic sensitivity and single-molecule spatial resolution. We demonstrate that this method enables multidimensional study of heterogeneous biomolecular systems over multiple length scales by nanoscopy and vibrational spectroscopy. We then illustrate the capabilities of this platform by capturing and analyzing the structural conformations of transient oligomeric species formed at the early stages of the self-assembly of α-synuclein, which are associated with the onset of Parkinson's disease.
Language	English
Publishing date	2023-01-13
Publishing country	United States
Document type	Journal Article
ZDB-ID	2810933-8
ISSN	2375-2548 ; 2375-2548
ISSN (online)	2375-2548
ISSN	2375-2548
DOI	10.1126/sciadv.abq3151
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Article: Type IIB von Willebrand disease: a paradox explains how von Willebrand factor works.

Ruggeri, Z M

Journal of thrombosis and haemostasis : JTH

2003 Volume 2, Issue 1, Page(s) 2–6

Abstract: Type IIB is a variant form of von Willebrand disease in which a structural abnormality of von Willebrand factor (VWF) causes enhanced binding to the platelet glycoprotein Ib receptor. As a consequence of this functional alteration, there is a decrease in ...

Abstract	Type IIB is a variant form of von Willebrand disease in which a structural abnormality of von Willebrand factor (VWF) causes enhanced binding to the platelet glycoprotein Ib receptor. As a consequence of this functional alteration, there is a decrease in the concentration of the largest VWF multimers in plasma, and the platelet count may be episodically decreased as a consequence of microaggregation. The net result is an apparent paradox, since the presence of a hyperfunctional adhesive molecule in blood causes a bleeding tendency. Here I recall how my colleagues and I managed to understand what goes on in these patients.
MeSH term(s)	Genetic Variation ; History, 20th Century ; Humans ; von Willebrand Diseases/blood ; von Willebrand Diseases/etiology ; von Willebrand Diseases/history ; von Willebrand Factor/genetics ; von Willebrand Factor/history ; von Willebrand Factor/physiology
Chemical Substances	von Willebrand Factor
Language	English
Publishing date	2003-07-14
Publishing country	England
Document type	Autobiography ; Biography ; Historical Article ; Journal Article
ZDB-ID	2112661-6
ISSN	1538-7836 ; 1538-7933
ISSN (online)	1538-7836
ISSN	1538-7933
DOI	10.1111/j.1538-7836.2003.00523.x
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Zs.MO 295: Show issues

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Article: Platelet and von Willebrand factor interactions at the vessel wall.

Ruggeri, Z M / Ruggeri, Zaverio M

Hamostaseologie

2004 Volume 24, Issue 1, Page(s) 1–11

Abstract: The process of platelet thrombus formation contributes to the haemostatic response that prevents excessive blood loss after tissue injury, but may become a life-threatening disease mechanism by causing the acute thrombotic occlusion of atherosclerotic ... ...

Abstract	The process of platelet thrombus formation contributes to the haemostatic response that prevents excessive blood loss after tissue injury, but may become a life-threatening disease mechanism by causing the acute thrombotic occlusion of atherosclerotic arteries. The participation of platelets in the formation of thrombi is centered on their adhesive properties and the ability to respond to stimuli with rapid activation. Platelet adhesion and activation are multifaceted and modulated by different environmental conditions, suggesting that it should be possible to obtain a selective pharmacological inhibition of the pathways more relevant to athero-thrombosis than to haemostasis. In particular, progress in understanding the structure and function of von Willebrand factor and the mechanisms that underlie its interactions with vascular surfaces and platelets can elucidate important differentiating aspects of normal haemostasis and pathological arterial thrombosis.
MeSH term(s)	Blood Platelets/physiology ; Humans ; Platelet Adhesiveness ; Receptors, Collagen/physiology ; Thrombosis/physiopathology ; von Willebrand Factor/physiology
Chemical Substances	Receptors, Collagen ; von Willebrand Factor
Language	English
Publishing date	2004-03-18
Publishing country	Germany
Document type	Journal Article ; Review
ZDB-ID	801512-0
ISSN	0720-9355
ISSN	0720-9355
DOI	10.1267/hamo04010001
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Article ; Online: Strategic infection prevention after genetically modified hematopoietic stem cell therapies: recommendations from the International Society for Cell & Gene Therapy Stem Cell Engineering Committee.

Cytotherapy

2024

Abstract: There is lack of guidance for immune monitoring and infection prevention after administration of ex vivo genetically modified hematopoietic stem cell therapies (GMHSCT). We reviewed current infection prevention practices as reported by providers ... ...

Abstract	There is lack of guidance for immune monitoring and infection prevention after administration of ex vivo genetically modified hematopoietic stem cell therapies (GMHSCT). We reviewed current infection prevention practices as reported by providers experienced with GMHSCTs across North America and Europe, and assessed potential immunologic compromise associated with the therapeutic process of GMHSCTs described to date. Based on these assessments, and with consensus from members of the International Society for Cell & Gene Therapy (ISCT) Stem Cell Engineering Committee, we propose risk-adapted recommendations for immune monitoring, infection surveillance and prophylaxis, and revaccination after receipt of GMHSCTs. Disease-specific and GMHSCT-specific considerations should guide decision making for each therapy.
Language	English
Publishing date	2024-02-20
Publishing country	England
Document type	Journal Article
ZDB-ID	2039821-9
ISSN	1477-2566 ; 1465-3249
ISSN (online)	1477-2566
ISSN	1465-3249
DOI	10.1016/j.jcyt.2024.02.005
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Zs.A 5601: Show issues

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Article: Von Willebrand factor, platelets and endothelial cell interactions.

Ruggeri, Z M

Journal of thrombosis and haemostasis : JTH

2001 Volume 1, Issue 7, Page(s) 1335–1342

Abstract: 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 ... ...

Abstract	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. The mechanism of interaction between the VWF A1 domain and glycoprotein Ibalpha has been elucidated in detail, bringing us closer to understanding how this adhesive bond can oppose the fluid dynamic effects of rapidly flowing blood contributing to platelet adhesion and activation. Moreover, novel findings have been obtained on the link between regulation of VWF multimer size and microvascular thrombosis. This progress in basic research has provided critical information to define with greater precision the role of VWF in vascular biology and pathology, including its possible involvement in the onset of atherosclerosis and its acute thrombotic complications.
MeSH term(s)	Animals ; Blood Platelets/metabolism ; Cell Adhesion ; Endothelium, Vascular/cytology ; Extracellular Matrix/metabolism ; Humans ; Microscopy, Confocal ; Models, Biological ; Protein Structure, Tertiary ; Thrombosis/metabolism ; von Willebrand Factor/physiology
Chemical Substances	von Willebrand Factor
Language	English
Publishing date	2001-12-08
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	2112661-6
ISSN	1538-7836 ; 1538-7933
ISSN (online)	1538-7836
ISSN	1538-7933
DOI	10.1046/j.1538-7836.2003.00260.x
Database	MEDical Literature Analysis and Retrieval System OnLINE

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