A5107494920- Connective tissue disorders research
- Heat shock proteins research
- Per- and polyfluoroalkyl substances research
- Protease and Inhibitor Mechanisms
- Bone health and osteoporosis research
- Bone fractures and treatments
- Neurogenetic and Muscular Disorders Research
University of Pavia
2024-2025
Perfluorinated compounds (PFAS) are well recognized toxic pollutants for humans, but if their effect is equally harmful healthy and fragile people unknown. Addressing this question represents a need ensuring global health wellbeing to all individuals in world facing the progressive increase of aging related diseases. This study aimed evaluate impact perfluorooctane sulfonate (PFOS), perfluorooctanoic acid (PFOA) perfluorohexanoic (PFHxA) exposure on development skeletal phenotype using...
The proof-of-principle of the therapeutic potential heat shock protein 47 (HSP47) for diseases characterized by defects in collagen I synthesis is here proved osteogenesis imperfecta (OI), a prototype disorders. Most OI mutations delay chains folding, increasing their exposure to post translational modifications that affect secretion and impact extracellular matrix fibrils assembly. As model, we used primary fibroblasts from individuals with defect prolyl-3-hydroxylation complex, since are...