|
|
|
题名
|
作者
|
年代
|
出处
|
被引量
|
| 1 | Gsα^(R201C)and estrogen reveal different subsets of bone marrow adiponectin expressing osteogenic cells显示文摘The Gsα/cAMP signaling pathway mediates the effect of a variety of hormones and factors that regulate the homeostasis of the post-natal skeleton.Hence,the dysregulated activity of Gsαdue to gain-of-function mutations (R201C/R201H)results in severe architectural and functional derangements of the entire bone/bone marrow organ.While the consequences of gain-of-function mutations of Gsαhave been extensively investigated in osteoblasts and in bone marrow osteoprogenitor cells at various differentiation stages,their effect in adipogenically-committed bone marrow stromal cells has remained unaddressed.We generated a mouse model with expression of Gsα^(R201C) driven by the Adiponectin(Adq)promoter.Adq-Gsα^(R201C) mice developed a complex combination of metaphyseal,diaphyseal and cortical bone changes.In the metaphysis,Gsα^(R201C) caused an early phase of bone resorption followed by bone deposition.Metaphyseal bone formation was sustained by cells that were traced by Adq-Cre and eventually resulted in a high trabecular bone mass phenotype.In the diaphysis,Gsα^(R201C),in combination with estrogen,triggered the osteogenic activity of Adq-Cre-targeted perivascular bone marrow stromal cells leading to intramedullary bone formation.Finally,consistent with the previously unnoticed presence of Adq-Cre-marked pericytes in intraosseous blood vessels,Gsα^(R201C) caused the development of a lytic phenotype that affected both cortical(increased porosity)and trabecular(tunneling resorption)bone.These results provide the first evidence that the Adq-cell network in the skeleton not only regulates bone resorption but also contributes to bone formation,and that the Gsα/cAMP pathway is a major modulator of both functions. | Biagio Palmisano Rossella Labella Samantha Donsante Cristina Remoli Emanuela Spica Ilenia Coletta Giorgia Farinacci Michele Dello Spedale Venti Isabella Saggio Marta Serafini Pamela Gehron Robey Alessandro Corsi Mara Riminucci | 2022 | Bone Research2022，10，4： | 1 |
| 2 | Birth position,accoucheur,and perineal outcomes:informing women about choices for vaginal birth显示文摘 | Shorten A Donsante J Shorten B | 2002 | Birth2002，29，1： | 1 |
| 3 | Observed incidence of tumorigenesis in long-term rodent studies of rAAV vectors显示文摘 | Donsante A Vogler C Muzyczka N | 2001 | Gene Ther2001，8，： | 1 |
| 4 | Molecular correlates of epilepsy in early diagnosed and treated Menkes disease 显示文摘 | Kaler SG Liew C J Donsante A | 2010 | J Inherit Metab Dis2010，33，5： | 1 |
| 5 | Translational read - through of a nonsense mutation in ATPTA impacts treatment outcome in Menkes disease 显示文摘 | Kaler SG Tang J Donsante A | 2009 | Ann Neurol2009，65，1： | 1 |
| 6 | Birth position,accoucheur,and peri- neal outcomes: informing women about choices for vaginal birth显示文摘 | Shorten A Donsante J Shorten B | 2002 | Birth2002，29，1： | 1 |
| 7 | Molecular correlates of epilepsy in early diagnosed and treated Menkes disease显示文摘 | Kaler SG Liew C J Donsante A | 2010 | J Inherit Metab Dis2010，33，5： | 1 |
| 8 | AAV vector integration sites in mouse hepatocellular carcinoma显示文摘 | DONSANTE A MILLER D G LI Y | 2007 | Science2007，317，5837： | 1 |
| 9 | Increased frequency of congenital heart defects in Menkes disease显示文摘 | Hicks JD Donsante A Pierson TM | | 0，，02： | 1 |
| 10 | Molecular correlates of epilepsy in early diagnosed and treated Menkes disease显示文摘 | Kaler SG Liew CJ Donsante A | 2010 | Inherit Metab Dis2010，33，5： | 1 |
| 11 | Differences in ATP7A gene expression underlie intrafamilial variability in Menkes disease/ occipital horn syndrome显示文摘 | Donsante A Tang J Godwin SC | 2007 | J Med Genet2007，44，8： | 1 |
| 12 | AAV vector integration sites in mouse hepatocellular carcinoma显示文摘 | Donsante A Miller DG Li Y | | 0，，5837： | 1 |
| 13 | Observed incidence of tumorigenesis in long-term rodent studies of rAAV vectors显示文摘 | Donsante A Vogler C Muzyczka N | 2001 | Gene Ther2001，8，： | 1 |
| 14 | AAV vector integration sites in mouse hepatocellular carcinoma 显示文摘 | Donsante A Miller D G Li Y | 2007 | Science2007，317，： | 1 |
| 15 | Favorably skewed X - inactiva- tion accounts for neurological sparing in female carriers of Menkes disease显示文摘 | Desai V Donsante A Swoboda K J | 2011 | Clin Genet2011，79，2： | 1 |
| 16 | Somatic mosaicism in Menkes disease suggests choroid plexus - mediated copper transport to the de- veloping brain 显示文摘 | Donsante A Johnson P Jansen LA | 2010 | Am J Med Genet A2010，152，10： | 1 |
您所在的机构暂未开通此部分的功能，请联系管理员