http://www.cnr.it/ontology/cnr/individuo/prodotto/ID11819
Skeletal progenitors and the GNAS gene: fibrous dysplasia of bone read through stem cells (Articolo in rivista)
- Type
- Label
- Skeletal progenitors and the GNAS gene: fibrous dysplasia of bone read through stem cells (Articolo in rivista) (literal)
- Anno
- 2010-01-01T00:00:00+01:00 (literal)
- Alternative label
Riminucci M, Gehron Robey P, Saggio I, Bianco P. (2010)
Skeletal progenitors and the GNAS gene: fibrous dysplasia of bone read through stem cells
in Journal of molecular endocrinology
(literal)
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- Riminucci M, Gehron Robey P, Saggio I, Bianco P. (literal)
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- Pagina fine
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- Rivista
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- Note
- ISI Web of Science (WOS) (literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
- Riminucci M, Saggio I, Bianco P Sapienza Universita' di Roma
Gehron Robey P, NIH
Saggio I IBPM CNR (literal)
- Titolo
- Skeletal progenitors and the GNAS gene: fibrous dysplasia of bone read through stem cells (literal)
- Abstract
- Activating mutations of the GNAS gene, which causes fibrous dysplasia of bone (FD), lead to remarkable changes in the
properties of skeletal progenitors, and it is these changes that mediate the pathological effect of this gene on bone. Mutated
skeletal stem cells lose the ability to differentiate into adipocytes, and to maintain in situ, and transfer heterotopically, the
hematopoietic microenvironment, leading to abnormal bone marrow histology in FD. They overexpress molecular effectors
of osteoclastogenesis, thus promoting inappropriate bone resorption leading to fragility of FD bone. They express the
phosphate-regulating hormone FGF-23 at normal levels, whose excess in the serum of FD patients correlates with the
massof osteogenic cells withinFDlesions, leading to osteomalacia and deformity of theFDbone, and revealing that bone is
an endocrine organ regulating renal handling of phosphate. Mechanisms of allelic selection and stem cell selection occur in
mutated skeletal stem cells and contribute to the inherent diversity and evolution over time in FD. The definition of the
etiological role of GNAS mutations marks the watershed between many decades of descriptive observation and the
definition of cellular and molecular mechanisms that would explain and hopefully allow for a cure for the disease. Placing
stem cells at center stage has permitted substantial advances in one decade, and promises more for the one to come. (literal)
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