Osteoblasts from a Mandibuloacral dysplasia patient induce human blood precursors to differentiate into active osteoclasts (Articolo in rivista)

Type
Label
  • Osteoblasts from a Mandibuloacral dysplasia patient induce human blood precursors to differentiate into active osteoclasts (Articolo in rivista) (literal)
Anno
  • 2011-01-01T00:00:00+01:00 (literal)
Alternative label
  • Avnet S, Pallotta R, Perut F, Baldini N, Pittis MG, Saponari A, Lucarelli E, Dozza B, Greggi T, Maraldi NM, Capanni C, Mattioli E, Columbaro M, Lattanzi G. (2011)
    Osteoblasts from a Mandibuloacral dysplasia patient induce human blood precursors to differentiate into active osteoclasts
    (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
  • Avnet S, Pallotta R, Perut F, Baldini N, Pittis MG, Saponari A, Lucarelli E, Dozza B, Greggi T, Maraldi NM, Capanni C, Mattioli E, Columbaro M, Lattanzi G. (literal)
Pagina inizio
  • 711 (literal)
Pagina fine
  • 718 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume
  • 1812 (literal)
Note
  • ISI Web of Science (WOS) (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
  • Laboratory for Pathophysiology, Istituto Ortopedico Rizzoli, Bologna, Italy; Clinical Genetics Department of Medicine and Aging Sciences, D'Annunzio University, Chieti, Italy; Rare Disease Coordination Center, University Hospital, Udine, Italy, Bone Regeneration Laboratory, Istituto Ortopedico Rizzoli, Bologna, Italy; Spinal Deformity Surgery Department, Istituto Ortopedico Rizzoli, Bologna, Italy; Laboratory of Musculoskeletal Cell Biology/RAMSES, Istituto Ortopedico Rizzoli, Bologna, Italy; Institute for Molecular Genetics, IGM-CNR, Unit of Bologna, Bologna, Italy (literal)
Titolo
  • Osteoblasts from a Mandibuloacral dysplasia patient induce human blood precursors to differentiate into active osteoclasts (literal)
Abstract
  • Mandibuloacral dysplasia type A (MADA) is a rare disease caused by mutations in the LMNA gene encoding A type lamins. Patients affected by MADA suffer from partial lipodystrophy, skin abnormalities and accelerated aging. Typical of MADA is also bone resorption at defined districts including terminal phalanges, mandible and clavicles. Little is known about the biological mechanism underlying osteolysis in MADA. In the reported study, we analyzed an osteoblast primary culture derived from the cervical vertebrae of a MADA patient bearing the homozygous R527H LMNA mutation. MADA osteoblasts showed nuclear abnormalities typical of laminopathic cells, but they proliferated in culture and underwent differentiation upon stimulation with dexametasone and beta-glycerophosphate. Differentiated osteoblasts showed proper production of bone mineral matrix until passage 8 in culture, suggesting a good differentiation activity. (literal)
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