139 results on '"Bezzerri, Valentino"'
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2. Growth Charts for Shwachman–Diamond Syndrome at Ages 0 to 18 Years.
3. The Molecular and Genetic Mechanisms of Inherited Bone Marrow Failure Syndromes: The Role of Inflammatory Cytokines in Their Pathogenesis
4. Does cystic fibrosis constitute an advantage in COVID-19 infection?
5. Shwachman-Diamond syndromes: clinical, genetic, and biochemical insights from the rare variants
6. Counteracting the Common Shwachman–Diamond Syndrome-Causing SBDS c.258+2T>C Mutation by RNA Therapeutics and Base/Prime Editing
7. Immunogenicity and Safety of the BNT162b2 COVID-19 Vaccine in Patients with Cystic Fibrosis with or without Lung Transplantation
8. Is cellular senescence involved in cystic fibrosis?
9. Study of Angiogenic Potential and Oxidative Metabolism in Mesenchymal Stromal Cells Derived from Shwachman-Diamond Syndrome Patients
10. 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
11. Novel Translational Read-through–Inducing Drugs as a Therapeutic Option for Shwachman-Diamond Syndrome
12. Enhanced p53 Levels Are Involved in the Reduced Mineralization Capacity of Osteoblasts Derived from Shwachman–Diamond Syndrome Subjects
13. Anti-inflammatory effect of miglustat in bronchial epithelial cells
14. Expression of microRNA-93 and Interleukin-8 during Pseudomonas aeruginosa-Mediated Induction of Proinflammatory Responses
15. Modulators of Sphingolipid Metabolism Reduce Lung Inflammation
16. Nonsense Suppression Therapy: New Hypothesis for the Treatment of Inherited Bone Marrow Failure Syndromes
17. 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
18. A Signature of Differentially Expressed Micrornas in Lymphoblastoid Cells from Shwachman-Diamond Syndrome Patients Indicates Possible Molecular Targets for Mirna Therapeutics
19. Breakthroughs in Preclinical Development of Ataluren (PTC124) As Therapeutic Option for Patients Affected By Shwachman-Diamond Syndrome: Towards the First Clinical Trial
20. Transcription Factor Oligodeoxynucleotides to NF-κB Inhibit Transcription of IL-8 in Bronchial Cells
21. MPB-07 Reduces the Inflammatory Response to Pseudomonas aeruginosa in Cystic Fibrosis Bronchial Cells
22. PLCB3 Loss-of-function Reduces P. aeruginosa-dependent IL-8 Release in Cystic Fibrosis
23. PLCB3 Loss of Function Reduces Pseudomonas aeruginosa–Dependent IL-8 Release in Cystic Fibrosis
24. Ataluren-driven restoration of Shwachman-Bodian-Diamond syndrome protein function in Shwachman-Diamond syndrome bone marrow cells
25. β-Sitosterol Reduces the Expression of Chemotactic Cytokine Genes in Cystic Fibrosis Bronchial Epithelial Cells
26. Differential Effects of Angelicin Analogues on NF-κB Activity and IL-8 Gene Expression in Cystic Fibrosis IB3-1 Cells
27. 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
28. Transient Receptor Potential Ankyrin 1 Channels Modulate Inflammatory Response in Respiratory Cells from Patients with Cystic Fibrosis
29. Additional file 1: Figure S1. of Regulation of IL-8 gene expression in gliomas by microRNA miR-93
30. Proinflammatory signal transduction in epithelial cells: the model of cystic fibrosis lung disease
31. New insights into the Shwachman-Diamond Syndrome-related haematological disorder: hyper-activation of mTOR and STAT3 in leukocytes
32. miRNA array screening reveals cooperative MGMT-regulation between miR-181d-5p and miR-409-3p in glioblastoma
33. Regulation of IL-8 gene expression in gliomas by microRNA miR-93
34. PSEUDOMONAS AERUGINOSA MODULATES THE PHOSPHORYLATION CHANGES OF SCAFFOLDING PROTEINS AND APICAL EXPRESSION OF CFTR IN AIRWAYS
35. IMINOSUGAR-BASED INHIBITORS OF CERAMIDE GLUCOSYL-TRANSFERASE (GLCCERT) AND NONLYSOSOMAL GLUCOSYLCERAMIDASE (GBA2) REDUCE THE INFLAMMATORY RESPONSE TO P. AERUGINOSA IN CF BRONCHIAL EPITHELIAL CELLS
36. PLCB3 cooperates with the Toll-like receptors' signaling cascade enhancing P.aeruginosa-dependent IL-8 expression in bronchial epithelial cells
37. Mitochondrial Ca2+-dependent NLRP3 activation exacerbates the Pseudomonas aeruginosa-driven inflammatory response in cystic fibrosis
38. EFFETTO ANTI-INFIAMMATORIO DI TRIMETIL-ANGELICINA SU CELLULE DELL'EPITELIO BRONCHIALE
39. Effetto anti-infiammatorio del miglustat su cellule dell'epitelio bronchiale
40. MPB-07 Reduces the Inflammatory Response to Pseudomonas aeruginosa in CF Bronchial Cells
41. GBA2-Encoded β-Glucosidase Activity Is Involved in the Inflammatory Response to Pseudomonas aeruginosa
42. Trimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells
43. Trimethylangelicin reduces IL-8 transcription and potentiates CFTR function
44. Docking of molecules identified in bioactive medicinal plants extracts into the p50 NF-kappaB transcription factor: correlation with inhibition of NF-kappaB/DNA interactions and inhibitory effects on IL-8 gene expression
45. Interaction of Adenovirus Type 5 Fiber with the Coxsackievirus and Adenovirus Receptor Activates Inflammatory Response in Human Respiratory Cells
46. 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
47. GBA2-Encoded β-Glucosidase Activity Is Involved in the Inflammatory Response to Pseudomonas aeruginosa.
48. Mitochondrial Ca2+-dependent NLRP3 activation exacerbates the Pseudomonas aeruginosa-driven inflammatory response in cystic fibrosis.
49. Novel Translational Read-through–Inducing Drugs as a Therapeutic Option for Shwachman-Diamond Syndrome
50. GBA2-Encoded β-Glucosidase Activity Is Involved in the Inflammatory Response to Pseudomonas aeruginosa.
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