46 results on '"Bezzerri, Valentino"'
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2. COVID‐19 disease in patients with chronic neutropenia: The experience from the European Network for Innovative Diagnosis and Treatment of Chronic Neutropenias
3. Growth Charts for Shwachman–Diamond Syndrome at Ages 0 to 18 Years.
4. Ataluren improves myelopoiesis and neutrophil chemotaxis by restoring ribosome biogenesis and reducing p53 levels in Shwachman–Diamond syndrome cells
5. The Molecular and Genetic Mechanisms of Inherited Bone Marrow Failure Syndromes: The Role of Inflammatory Cytokines in Their Pathogenesis
6. Shwachman-Diamond Syndrome: Molecular Mechanisms and Current Perspectives
7. Does cystic fibrosis constitute an advantage in COVID-19 infection?
8. The pathogenic variant c.258+533_459+403del in SBDS gene is the commonest rare variant in Italy and shows a founder effect
9. Shwachman-Diamond syndromes: clinical, genetic, and biochemical insights from the rare variants
10. Counteracting the Common Shwachman–Diamond Syndrome-Causing SBDS c.258+2T>C Mutation by RNA Therapeutics and Base/Prime Editing
11. Immunogenicity and Safety of the BNT162b2 COVID-19 Vaccine in Patients with Cystic Fibrosis with or without Lung Transplantation
12. Is cellular senescence involved in cystic fibrosis?
13. Study of Angiogenic Potential and Oxidative Metabolism in Mesenchymal Stromal Cells Derived from Shwachman-Diamond Syndrome Patients
14. Deletion of eif6 Partially Rescues Survival, but Not Neutropenia, of Zebrafish Model of Shwachman-Diamond Syndrome: The Roles of Sbds and Eif6 in Perturbing Lipid Metabolism
15. Novel Translational Read-through–Inducing Drugs as a Therapeutic Option for Shwachman-Diamond Syndrome
16. Pseudomonas aeruginosa reduces the expression of CFTR via post-translational modification of NHERF1
17. Enhanced p53 Levels Are Involved in the Reduced Mineralization Capacity of Osteoblasts Derived from Shwachman–Diamond Syndrome Subjects
18. ACE2 expression and localization are regulated by CFTR: implications beyond cystic fibrosis
19. Does Cystic Fibrosis Constitute an Advantage in COVID-19 Infection?
20. Nonsense Suppression Therapy: New Hypothesis for the Treatment of Inherited Bone Marrow Failure Syndromes
21. mTOR and STAT3 Pathway Hyper-Activation is Associated with Elevated Interleukin-6 Levels in Patients with Shwachman-Diamond Syndrome: Further Evidence of Lymphoid Lineage Impairment
22. A Signature of Differentially Expressed Micrornas in Lymphoblastoid Cells from Shwachman-Diamond Syndrome Patients Indicates Possible Molecular Targets for Mirna Therapeutics
23. Breakthroughs in Preclinical Development of Ataluren (PTC124) As Therapeutic Option for Patients Affected By Shwachman-Diamond Syndrome: Towards the First Clinical Trial
24. PLCB3 Loss-of-function Reduces P. aeruginosa-dependent IL-8 Release in Cystic Fibrosis
25. Peripheral blood immunophenotyping in a large cohort of patients with Shwachman–Diamond syndrome
26. Shwachman-Diamond Syndrome: Molecular Mechanisms and Current Perspectives
27. PLCB3 Loss of Function Reduces Pseudomonas aeruginosa–Dependent IL-8 Release in Cystic Fibrosis
28. Ataluren-driven restoration of Shwachman-Bodian-Diamond syndrome protein function in Shwachman-Diamond syndrome bone marrow cells
29. An antisense peptide nucleic acid against Pseudomonas aeruginosa inhibiting bacterial-induced inflammatory responses in the cystic fibrosis IB3-1 cellular model system
30. β-Sitosterol Reduces the Expression of Chemotactic Cytokine Genes in Cystic Fibrosis Bronchial Epithelial Cells
31. Differential Effects of Angelicin Analogues on NF-κB Activity and IL-8 Gene Expression in Cystic Fibrosis IB3-1 Cells
32. Further Insights into the Hematological Disorders Observed in Shwachman-Diamond Syndrome: mTOR and STAT3 Hyper-Phosphorylation in CD4+ and CD8+ T Cells and Efficacy of Rapamycin Treatment
33. Transient Receptor Potential Ankyrin 1 Channels Modulate Inflammatory Response in Respiratory Cells from Patients with Cystic Fibrosis
34. Additional file 1: Figure S1. of Regulation of IL-8 gene expression in gliomas by microRNA miR-93
35. Antioxidants As a Novel Treatment to Revert Impaired Angiogenic Potential Driven By Metabolic Alterations Observed in Shwachman-Diamond Syndrome Derived Mesenchymal Stromal Cells
36. New insights into the Shwachman-Diamond Syndrome-related haematological disorder: hyper-activation of mTOR and STAT3 in leukocytes
37. miRNA array screening reveals cooperative MGMT-regulation between miR-181d-5p and miR-409-3p in glioblastoma
38. Regulation of IL-8 gene expression in gliomas by microRNA miR-93
39. Mitochondrial Ca2+-dependent NLRP3 activation exacerbates the Pseudomonas aeruginosa-driven inflammatory response in cystic fibrosis
40. Antibacterial and anti-inflammatory activity of a temporin B peptide analogue on an in vitro model of cystic fibrosis.
41. Deletion of eif6Partially Rescues Survival, but Not Neutropenia, of Zebrafish Model of Shwachman-Diamond Syndrome: The Roles of Sbds and Eif6 in Perturbing Lipid Metabolism
42. Mitochondrial Ca2+-dependent NLRP3 activation exacerbates the Pseudomonas aeruginosa-driven inflammatory response in cystic fibrosis.
43. Novel Translational Read-through–Inducing Drugs as a Therapeutic Option for Shwachman-Diamond Syndrome
44. miRNA array screening reveals cooperative MGMT-regulation between miR-181d-5p and miR-409-3p in glioblastoma
45. Ataluren improves myelopoiesis and neutrophil chemotaxis by restoring ribosome biogenesis and reducing p53 levels in Shwachman-Diamond syndrome cells.
46. Differential Effects of Angelicin Analogues on NF- κ B Activity and IL-8 Gene Expression in Cystic Fibrosis IB3-1 Cells.
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