A5080470691- Bone health and treatments
- Bone Tumor Diagnosis and Treatments
- Oral and Maxillofacial Pathology
- Hematopoietic Stem Cell Transplantation
- CAR-T cell therapy research
- Immune Cell Function and Interaction
- Bone Metabolism and Diseases
- Hematological disorders and diagnostics
- Acute Myeloid Leukemia Research
- Mesenchymal stem cell research
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- MicroRNA in disease regulation
- TGF-β signaling in diseases
- 3D Printing in Biomedical Research
- Cytokine Signaling Pathways and Interactions
- Fibroblast Growth Factor Research
- Lysosomal Storage Disorders Research
- Connective tissue disorders research
- Bone and Joint Diseases
- Soft tissue tumor case studies
- Pain Mechanisms and Treatments
- Genetic Syndromes and Imprinting
- dental development and anomalies
- Mosquito-borne diseases and control
- Peptidase Inhibition and Analysis
Sapienza University of Rome
2016-2024
Istituti di Ricovero e Cura a Carattere Scientifico
2023-2024
University of Milano-Bicocca
2021
Abstract Background Paediatric acute myeloid leukaemia (AML) is characterized by poor outcomes in patients with relapsed/refractory disease, despite the improvements intensive standard therapy. The leukaemic cells of paediatric AML show high expression CD123 antigen, and this finding provides biological basis to target chimeric antigen receptor (CAR). However, CAR.CD123 therapy hampered on-target off-tumour toxicity a long “vein-to-vein” time. Methods We developed an off-the-shelf product...
Acute myeloid leukemia (AML) is a hematological malignancy derived from neoplastic progenitor cells characterized by abnormal clonal proliferation and differentiation. Although novel therapeutic strategies have recently been introduced, the prognosis of AML remains unsatisfactory. So far, efficacy chimeric antigen receptor (CAR)-T therapy in has hampered several factors including poor accumulation blood-injected bone marrow (BM) niche, where chemotherapy-resistant leukemic stem reside. Thus,...
Multipotent stromal cells are considered attractive sources for cell therapy and tissue engineering. Despite numerous experimental clinical studies, broad application of therapeutics is not yet emerging. A major challenge the functional diversity available sources. Here, we investigated regenerative potential clinically relevant human from bone marrow (BMSCs), white adipose tissue, umbilical cord compared with mature chondrocytes skin fibroblasts in vitro vivo. Although all types could...
Fibrous dysplasia of bone/McCune-Albright syndrome (Polyostotic FD/MAS; OMIM#174800) is a crippling skeletal disease caused by gain-of-function mutations Gs α. Enhanced bone resorption recurrent histological feature FD and major cause fragility affected bones. Previous work suggests that increased in driven RANKL some studies have shown the anti-RANKL monoclonal antibody, denosumab, reduces turnover pain patients. However, effect inhibition on histopathology its impact natural history remain...
Brain derived neurotrophic factor (BDNF) is a neurotrophin, expressed in the central nervous system and peripheral tissues, that regulated by Gsα/cAMP pathway. In bone, it regulates osteogenesis stimulates RANKL secretion osteoclast formation osteolytic tumors such as Multiple Myeloma. Fibrous dysplasia (FD) of bone rare genetic disease skeleton caused gain-of-function mutations Gsα gene which RANKL-dependent enhanced resorption major cause fragility clinical morbidity. We observed BDNF...
The Gsα/cAMP signaling pathway mediates the effect of a variety hormones and factors that regulate homeostasis post-natal skeleton. Hence, dysregulated activity Gsα due to gain-of-function mutations (R201C/R201H) results in severe architectural functional derangements entire bone/bone marrow organ. While consequences have been extensively investigated osteoblasts bone osteoprogenitor cells at various differentiation stages, their adipogenically-committed stromal has remained unaddressed. We...
ABSTRACT Human umbilical cord blood (CB) has attracted much attention as a reservoir for functional hematopoietic stem and progenitor cells, and, recently, source of blood-borne fibroblasts (CB-BFs). Previously, we demonstrated that bone marrow stromal cell (BMSC) CB-BF pellet cultures make cartilage in vitro. Furthermore, upon vivo transplantation, BMSC pellets remodelled into miniature bone/marrow organoids. Using this model, asked whether populations express characteristics the (HSC)...
Terminally differentiated cells are defined by their inability to proliferate. When forced re-enter the cell cycle, they generally cannot undergo long-term replication. Our previous work with myotubes has shown that these fail proliferate because of intrinsic complete DNA Moreover, we have reported pronounced modifications deoxynucleotide metabolism during myogenesis. Here investigate causes incomplete duplication in cycle-reactivated (rMt). We find rMt possess extremely low levels thymidine...
Dysostosis multiplex is a major cause of morbidity in Hurler syndrome (mucopolysaccharidosis type IH [MPS IH], OMIM #607014) because currently available therapies have limited success its prevention and reversion. Unfortunately, the elucidation skeletal pathogenesis MPS by difficulties obtaining bone specimens from pediatric patients poor reproducibility animal models. Thus, application experimental systems that can be used to dissect cellular molecular mechanisms underlying phenotype...
Acute myeloid leukemia (AML) is a highly heterogeneous malignancy caused by various genetic alterations and characterized the accumulation of immature blasts in bone marrow (BM). This abnormal growth AML cells disrupts normal hematopoiesis alters BM microenvironment components, establishing niche supportive leukemogenesis. Bone stromal (BMSCs) play pivotal role giving rise to essential elements niche, including adipocytes osteogenic cells. Animal models have shown that significantly...
Bone, cartilage, and marrow adipocytes are generated by skeletal progenitors, but the relationships between lineages mechanisms controlling their differentiation poorly understood. We established mouse clonal progenitors with distinct properties analyzed transcriptome. Unipotent osteogenic adipogenic cells expressed specific transcriptional programs, whereas bipotent clones combined expression of those genes did not show a unique signature. tested potential regulators lineage commitment...
Effects of oxygen tension on the generation, expansion, proliferation and differentiation stromal cell types is widely described in literature. However, data internal heterogeneity applied populations at different O2 levels possible impacts potentials are controversial. Here, expression 39 human HOX genes was determined neonatal cord blood cells linked to differentiation-associated signatures. In blood, unrestricted somatic (USSCs), lacking gene expression, blood-derived multipotent...
We compared the effects of a nitrogen-containing bisphosphonate (N-BP), zoledronic acid (ZA), and an anti-mouse RANKL antibody (anti-mRANKL Ab) on bone tissue pathology transgenic mouse model human fibrous dysplasia (FD). For comparison, we also reviewed histological samples child with McCune-Albright syndrome (MAS) treated Pamidronate for 3 years. EF1α-GsαR201C mice FD-like lesions in tail vertebrae were either 0.2 mg/kg ZA at day 0, 7, 14 or 300 μg/mouse anti-mRANKL Ab 0 21. All monitored...
Identifying the molecular networks that underlie Fibrous Dysplasia (FD) is key to understand pathogenesis of disease, refine current diagnostic approaches and develop efficacious therapies. In this study, we used NanoString nCounter Analysis System investigate gene signature a series nine Formalin Fixed Decalcified Paraffin-Embedded (FFDPE) bone biopsies from seven FD patients. We analyzed expression level 770 genes. Unsupervised clustering analysis demonstrated partitioning into two...
The bone marrow adipose tissue constitutes more than two-thirds of the volume in adult life and is known to have unique metabolic functional properties. In neoplastic disorders, adipocytes (BMAds) contribute create a favorable microenvironment survival proliferation cancer cells. Many studies explored molecular crosstalk between BMAds cells, predominantly ex-vivo experimental systems or animal models. However, little on features human marrow. aim our study was analyze situ changes morphology...
Recent evidence suggests that the bone marrow (BM) plays a key role in diffusion of P. falciparum malaria by providing "niche" for maturation parasite gametocytes, responsible human-to-mosquito transmission. Suitable humanized vivo models to study mechanisms interplay between and human BM components are still missing. We report novel experimental system based on infusion immature gametocytes into immunocompromised mice carrying chimeric ectopic ossicles whose stromal compartments derive from...
Only 0.3–1% of laryngeal cancer are non-squamous cell neoplasms. Of these, a rare entity is inflammatory myofibroblastic tumour (IMT), in which anaplastic lymphoma kinase-1 (ALK-1) frequently expressed. Just 50 cases IMT have been reported. Therefore, many otolaryngologists may be unfamiliar with this type and prone to its over- or undertreatment. We report case ALK-1–negative treated transoral endoscopic excision disease-free 6 months after surgery.
Abstract Bone pain is a major symptom of many skeletal disorders. Fibrous dysplasia (FD) genetic disease with mono or polyostotic phenotype due to the post-zygotic occurrence causative Gsα mutation. in FD often associates deformities and fractures nerve impingement by pathological tissue. However, even absence complications, patients complain chronic that does not correlate their burden. Multiple hypotheses have been made explain this pain. its pathogenetic mechanisms remain, as yet, largely...