http://www.cnr.it/ontology/cnr/individuo/prodotto/ID314079
Modulation of TGFbeta 2 levels by lamin A in U2-OS osteoblast-like cells: understanding the osteolytic process triggered by altered lamins (Articolo in rivista)
- Type
- Label
- Modulation of TGFbeta 2 levels by lamin A in U2-OS osteoblast-like cells: understanding the osteolytic process triggered by altered lamins (Articolo in rivista) (literal)
- Anno
- 2015-01-01T00:00:00+01:00 (literal)
- Alternative label
Camilla Evangelisti1 , Pia Bernasconi2 , Paola Cavalcante2, Cristina Cappelletti2,
Maria Rosaria D'Apice3, Paolo Sbraccia4, Giuseppe Novelli5, Sabino Prencipe1,
Silvia Lemma6, Nicola Baldini6, Sofia Avnet6, Stefano Squarzoni1, Alberto M.
Martelli7, Giovanna Lattanzi1 (2015)
Modulation of TGFbeta 2 levels by lamin A in U2-OS osteoblast-like cells: understanding the osteolytic process triggered by altered lamins
in Oncotarget
(literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
- Camilla Evangelisti1 , Pia Bernasconi2 , Paola Cavalcante2, Cristina Cappelletti2,
Maria Rosaria D'Apice3, Paolo Sbraccia4, Giuseppe Novelli5, Sabino Prencipe1,
Silvia Lemma6, Nicola Baldini6, Sofia Avnet6, Stefano Squarzoni1, Alberto M.
Martelli7, Giovanna Lattanzi1 (literal)
- Rivista
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
- 1 Rizzoli Orthopedic Institute, Laboratory of Musculoskeletal Cell Biology and CNR Institute for Molecular Genetics, Unit of
Bologna, Bologna, Italy
2Neurology IV Unit - Neuroimmunology and Neuromuscular Disorders, Foundation IRCCS Neurological Institute \"Carlo
Besta\", Milan, Italy
3U.O.C. Medical Genetics Laboratory, AOU Policlinico Tor Vergata, Rome, Italy
4Department of Internal Medicine, University of Rome Tor Vergata, Rome, Italy
5Department of Biomedicine and Prevention, University of Rome Tor Vergata, Rome, Italy
6Rizzoli Orthopedic Institute, Laboratory for Pathophysiology, Bologna, Italy
7Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy (literal)
- Titolo
- Modulation of TGFbeta 2 levels by lamin A in U2-OS osteoblast-like cells: understanding the osteolytic process triggered by altered lamins (literal)
- Abstract
- Transforming growth factor beta (TGFbeta) plays an essential role in bone
homeostasis and deregulation of TGFbeta occurs in bone pathologies. Patients
affected by Mandibuloacral Dysplasia (MADA), a progeroid disease linked to LMNA
mutations, suffer from an osteolytic process. Our previous work showed that MADA
osteoblasts secrete excess amount of TGFbeta 2, which in turn elicits differentiation
of human blood precursors into osteoclasts. Here, we sought to determine how altered
lamin A affects TGFbeta signaling. Our results show that wild-type lamin A negatively
modulates TGFbeta 2 levels in osteoblast-like U2-OS cells, while the R527H mutated
prelamin A as well as farnesylated prelamin A do not, ultimately leading to increased
secretion of TGFbeta 2. TGFbeta 2 in turn, triggers the Akt/mTOR pathway and
upregulates osteoprotegerin and cathepsin K. TGFbeta 2 neutralization rescues Akt/
mTOR activation and the downstream transcriptional effects, an effect also obtained
by statins or RAD001 treatment. Our results unravel an unexpected role of lamin A
in TGFbeta 2 regulation and indicate rapamycin analogs and neutralizing antibodies
to TGFbeta 2 as new potential therapeutic tools for MADA. (literal)
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