A5003983960- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Platelet Disorders and Treatments
- Chronic Myeloid Leukemia Treatments
- Eosinophilic Disorders and Syndromes
- Acute Myeloid Leukemia Research
- Cell Adhesion Molecules Research
- Antiplatelet Therapy and Cardiovascular Diseases
- Blood properties and coagulation
- Proteoglycans and glycosaminoglycans research
- Kruppel-like factors research
- Hematopoietic Stem Cell Transplantation
- Erythrocyte Function and Pathophysiology
- Immune cells in cancer
- Hematological disorders and diagnostics
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Boron and Carbon Nanomaterials Research
- Boron Compounds in Chemistry
- Inflammatory Biomarkers in Disease Prognosis
- Advanced Glycation End Products research
- Pancreatic function and diabetes
- Silk-based biomaterials and applications
- Galectins and Cancer Biology
- Collagen: Extraction and Characterization
- Protein Interaction Studies and Fluorescence Analysis
- Renal Diseases and Glomerulopathies
University of Pavia
2016-2025
Magna Graecia University
2007-2025
Tufts University
2025
Istituti di Ricovero e Cura a Carattere Scientifico
2013-2022
Policlinico San Matteo Fondazione
2014-2018
A quarter of patients with essential thrombocythemia or primary myelofibrosis carry a driver mutation CALR, the calreticulin gene. 52-bp deletion (type 1) and 5-bp insertion 2 mutation) are most frequent variants. These indels might differentially impair calcium binding activity mutant calreticulin. We studied relationship between subtype biological/clinical features disease. Thirty-two different types CALR variants were identified in 311 patients. Based on their predicted effect C-terminal,...
Abstract Mechanisms through which mature megakaryocytes (Mks) and their progenitors sense the bone marrow extracellular matrix to promote lineage differentiation in health disease are still partially understood. We found PIEZO1, a mechanosensitive cation channel, be expressed mouse human Mks. Human mutations PIEZO1 have been described associated with blood cell disorders. Yet, role for megakaryopoiesis proplatelet formation has never investigated. Here, we show that activation of increases...
Megakaryocytes (MK) in the bone marrow (BM) are immersed a network of extracellular matrix components that regulates platelet release into circulation. Combining biological and bioengineering approaches, we found activation transient receptor potential cation channel subfamily V member 4 (TRPV4), mechano-sensitive ion channel, is induced upon MK adhesion on softer matrices. This response promoted production by triggering cascade events lead to calcium influx, β1 integrin internalization, Akt...
Extracellular matrix (ECM) components initiate crucial biochemical and biomechanical cues that are required for bone marrow homeostasis. In our research, we prove a peri-cellular composed primarily of type III IV collagens, fibronectin surrounds human megakaryocytes in the marrow. The data collected support hypothesis possess complete mechanism to synthesize ECM components, thrombopoietin is pivotal regulator this new function inducing transforming growth factor-β1 (TGF-β1) release...
The fibronectin EDA isoform (EDA FN) is instrumental in fibrogenesis but, to date, its expression and function bone marrow (BM) fibrosis have not been explored. We found that mice constitutively expressing the domain (EIIIA+/+), but knockout mice, are more prone develop BM upon treatment with thrombopoietin (TPO) mimetic romiplostim (TPOhigh). Mechanistically, FN binds TLR4 sustains progenitor cell proliferation megakaryopoiesis a TPO-independent fashion, inducing LPS-like responses, such as...
This series of illustrated capsules summarizes the presentations made by speakers at first International Advanced Course in Platelet Research held Murcia (Spain) from 27 to 28 September, 2024. is course receive a Fundamental Workshop Grant Society on Thrombosis and Haemostasis (ISTH) was also supported administratively scientifically Spanish (SETH). unique focused new methodologies applied platelet research how these are increasing our understanding formation, their multifunctionality...
Summary Tissue factor (TF), the main activator of blood coagulation cascade, has been shown to be expressed by platelets. Despite evidence that both megakaryocytes and platelets express TF mRNA, can make de novo protein synthesis, mechanism thought responsible for presence within is through uptake positive microparticles. In this study we assessed 1) whether human synthesise transfer it 2) contribution platelet-TF platelet hemostatic capacity. order avoid cross-talk with circulating...
Endoplasmic reticulum stress is an emerging significant player in the molecular pathology of connective tissue disorders. In response to endoplasmic stress, cells can upregulate macroautophagy/autophagy, a fundamental cellular homeostatic process used by degrade and recycle proteins or remove damaged organelles. these scenarios, autophagy activation support cell survival. Here we demonstrated vitro vivo approaches that megakaryocytes derived from col6a1−⁄− (collagen, type VI, alpha 1) null...
Abstract Glycosylation is recognized as a key process for proper megakaryopoiesis and platelet formation. The enzyme uridine diphosphate (UDP)-galactose-4-epimerase, encoded by GALE, involved in galactose metabolism protein glycosylation. Here, we studied 3 patients from 2 unrelated families who showed lifelong severe thrombocytopenia, bleeding diathesis, mental retardation, mitral valve prolapse, jaundice. Whole-exome sequencing revealed 4 variants that affect of those previously unreported...