Cargando…

A p.Arg127Gln variant in GPIbα LRR5 allosterically enhances affinity for VWF: a novel form of platelet-type VWD

Gain-of-function (GOF) variants in GP1BA cause platelet-type von Willebrand disease (PT-VWD), a rare inherited autosomal dominant bleeding disorder characterized by enhanced platelet GPIbα to von Willebrand factor (VWF) interaction, and thrombocytopenia. To date, only 6 variants causing PT-VWD have...

Descripción completa

Detalles Bibliográficos
Autores principales:	Bury, Loredana, Falcinelli, Emanuela, Kuchi Bhotla, Haripriya, Mezzasoma, Anna Maria, Guglielmini, Giuseppe, Tischer, Alexander, Moon-Tasson, Laurie, Auton, Matthew, Gresele, Paolo
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	American Society of Hematology 2022
Materias:	Platelets and Thrombopoiesis
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9006298/ https://www.ncbi.nlm.nih.gov/pubmed/34619770 http://dx.doi.org/10.1182/bloodadvances.2021005463

Ejemplares similares

Laboratory assays of VWF activity and use of desmopressin trials in the diagnosis of VWD: a systematic review and meta-analysis
por: Kalot, Mohamad A., et al.
Publicado: (2022)

Deletion of platelet CLEC-2 decreases GPIbα-mediated integrin αIIbβ3 activation and decreases thrombosis in TTP
por: Shao, Bojing, et al.
Publicado: (2022)

Defective RAB31-mediated megakaryocytic early endosomal trafficking of VWF, EGFR, and M6PR in RUNX1 deficiency
por: Jalagadugula, Gauthami, et al.
Publicado: (2022)

Expanding the genetic spectrum of TUBB1-related thrombocytopenia
por: Palma-Barqueros, Verónica, et al.
Publicado: (2021)

Functional genomics in zebrafish permits rapid characterization of novel platelet membrane proteins
por: O'Connor, Marie N., et al.
Publicado: (2009)

The tyrosine phosphatase CD148 is an essential positive regulator of platelet activation and thrombosis
por: Senis, Yotis A., et al.
Publicado: (2009)

Platelet gene expression and function in patients with COVID-19
por: Manne, Bhanu Kanth, et al.
Publicado: (2020)

COVID-19 concerns aggregate around platelets
por: Battinelli, Elisabeth M.
Publicado: (2020)

Platelet activation and platelet-monocyte aggregate formation trigger tissue factor expression in patients with severe COVID-19
por: Hottz, Eugenio D., et al.
Publicado: (2020)

Antibody-induced procoagulant platelets in severe COVID-19 infection
por: Althaus, Karina, et al.
Publicado: (2021)

Upregulation of cAMP prevents antibody-mediated thrombus formation in COVID-19
por: Zlamal, Jan, et al.
Publicado: (2022)

Low-dose decitabine modulates T-cell homeostasis and restores immune tolerance in immune thrombocytopenia
por: Han, Panpan, et al.
Publicado: (2021)

Aberrant glycosylation of anti-SARS-CoV-2 spike IgG is a prothrombotic stimulus for platelets
por: Bye, Alexander P., et al.
Publicado: (2021)

SARS-CoV-2 vaccination and ITP in patients with de novo or preexisting ITP
por: Lee, Eun-Ju, et al.
Publicado: (2022)

Insights in ChAdOx1 nCoV-19 vaccine-induced immune thrombotic thrombocytopenia
por: Greinacher, Andreas, et al.
Publicado: (2021)

Sugar and spike: not so nice
por: McKenzie, Steven E.
Publicado: (2021)

Iron status influences the response of cord blood megakaryocyte progenitors to eltrombopag in vitro
por: Liu, Zhi-Jian, et al.
Publicado: (2021)

The RNA-binding protein SRSF3 has an essential role in megakaryocyte maturation and platelet production
por: Heazlewood, Shen Y., et al.
Publicado: (2022)

Platelet activation and partial desensitization are associated with viral xenophagy in patients with severe COVID-19
por: Garcia, Cédric, et al.
Publicado: (2022)

Analysis of preplatelets and their barbell platelet derivatives by imaging flow cytometry
por: Kemble, Samuel, et al.
Publicado: (2022)

Platelet SHARPIN regulates platelet adhesion and inflammatory responses through associations with αIIbβ3 and LUBAC
por: Kasirer-Friede, Ana, et al.
Publicado: (2022)

Platelet-monocyte interaction amplifies thromboinflammation through tissue factor signaling in COVID-19
por: Hottz, Eugenio D., et al.
Publicado: (2022)

Platelet activation by SARS-CoV-2 implicates the release of active tissue factor by infected cells
por: Puhm, Florian, et al.
Publicado: (2022)

The interaction between anti-PF4 antibodies and anticoagulants in vaccine-induced thrombotic thrombocytopenia
por: Singh, Anurag, et al.
Publicado: (2022)

G protein–coupled receptor kinase 5 regulates thrombin signaling in platelets via PAR-1
por: Downes, Kate, et al.
Publicado: (2022)

COVID-19 vaccination in patients with immune thrombocytopenia
por: Visser, Chantal, et al.
Publicado: (2022)

Evans syndrome in adults: an observational multicenter study
por: Fattizzo, Bruno, et al.
Publicado: (2021)

Cost-effectiveness of eltrombopag vs intravenous immunoglobulin for the perioperative management of immune thrombocytopenia
por: Kaur, Manraj N., et al.
Publicado: (2022)

Multiparameter phenotyping of platelet reactivity for stratification of human cohorts
por: Dunster, Joanne L., et al.
Publicado: (2021)

Blood–brain barrier permeability in survivors of immune-mediated thrombotic thrombocytopenic purpura: a pilot study
por: Huang, Shih-Han Susan, et al.
Publicado: (2021)

A novel mechanism of thrombocytopenia by PS exposure through TMEM16F in sphingomyelin synthase 1 deficiency
por: Fujii, Yoritaka, et al.
Publicado: (2021)

Abnormalities of bone marrow B cells and plasma cells in primary immune thrombocytopenia
por: Yu, Tian-shu, et al.
Publicado: (2021)

Platelet variability index: a measure of platelet count fluctuations in patients with immune thrombocytopenia
por: Li, Na, et al.
Publicado: (2021)

Bone marrow remodeling supports hematopoiesis in response to immune thrombocytopenia progression in mice
por: Herd, Oliver J., et al.
Publicado: (2021)

Ablation of collagen VI leads to the release of platelets with altered function
por: Abbonante, Vittorio, et al.
Publicado: (2021)

Assessment of romiplostim immunogenicity in pediatric patients in clinical trials and in a global postmarketing registry
por: Bowers, Charles, et al.
Publicado: (2021)

Platelet FcγRIIA-induced serotonin release exacerbates the severity of transfusion-related acute lung injury in mice
por: El Mdawar, Marie-Belle, et al.
Publicado: (2021)

Heparin or nonheparin anticoagulants for VITT
por: Arnold, Donald M.
Publicado: (2022)

Megakaryocyte/platelet-derived TGF-β1 inhibits megakaryopoiesis in bone marrow by regulating thrombopoietin production in liver
por: Gostynska, Sandra, et al.
Publicado: (2022)

GPVI expression is linked to platelet size, age, and reactivity
por: Veninga, Alicia, et al.
Publicado: (2022)

Cannot write session to /tmp/vufind_sessions/sess_0ou198l60rkkog3tgjtgbnest9