Stimulation of Treg Cells to Inhibit Osteoclastogenesis in Gorham-Stout Disease
Gorham-Stout disease (GSD) is a very rare syndrome displaying excessive bone erosion and vascular lesion. Due to the rarity of the disease and to the limited studies, its etiopathogenesis is not entirely known. The involvement of immune system in the progressive osteolysis was recently suggested. In...
Main Authors: | , , , , , , , , , , , , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
Frontiers Media S.A.
2021-08-01
|
Series: | Frontiers in Cell and Developmental Biology |
Subjects: | |
Online Access: | https://www.frontiersin.org/articles/10.3389/fcell.2021.706596/full |
id |
doaj-8e80db9bdf524641bf6e3a6e976142f7 |
---|---|
record_format |
Article |
spelling |
doaj-8e80db9bdf524641bf6e3a6e976142f72021-09-03T11:48:31ZengFrontiers Media S.A.Frontiers in Cell and Developmental Biology2296-634X2021-08-01910.3389/fcell.2021.706596706596Stimulation of Treg Cells to Inhibit Osteoclastogenesis in Gorham-Stout DiseaseMichela Rossi0Ippolita Rana1Paola Sabrina Buonuomo2Giulia Battafarano3Viviana De Martino4Matteo D’Agostini5Ottavia Porzio6Ottavia Porzio7Cristiana Cipriani8Salvatore Minisola9Rita De Vito10Davide Vecchio11Michaela Veronika Gonfiantini12Alessandro Jenkner13Andrea Bartuli14Andrea Del Fattore15Bone Physiopathology Research Unit, Genetics and Rare Diseases Research Division, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyRare Diseases and Medical Genetic Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyRare Diseases and Medical Genetic Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyBone Physiopathology Research Unit, Genetics and Rare Diseases Research Division, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyDepartment of Clinical, Internal, Anaesthesiological and Cardiovascular Sciences, Sapienza University, Rome, ItalyClinical Laboratory, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyClinical Laboratory, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyDepartment of Experimental Medicine, University of Rome Tor Vergata, Rome, ItalyDepartment of Clinical, Internal, Anaesthesiological and Cardiovascular Sciences, Sapienza University, Rome, ItalyDepartment of Clinical, Internal, Anaesthesiological and Cardiovascular Sciences, Sapienza University, Rome, ItalyDepartment of Histopathology, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyRare Diseases and Medical Genetic Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyRare Diseases and Medical Genetic Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyDivision of Immunology and Infectious Diseases, Department of Pediatrics, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyRare Diseases and Medical Genetic Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyBone Physiopathology Research Unit, Genetics and Rare Diseases Research Division, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyGorham-Stout disease (GSD) is a very rare syndrome displaying excessive bone erosion and vascular lesion. Due to the rarity of the disease and to the limited studies, its etiopathogenesis is not entirely known. The involvement of immune system in the progressive osteolysis was recently suggested. Indeed, extensive reciprocal interactions between the immune and skeletal systems have been demonstrated. This study aimed to evaluate alterations of immune cells in GSD. An increase of CD8+ cells and reduction of CD4+ and CD4+CD25+CD127low cells was revealed in patients. Interestingly, patients’ regulatory T cells maintain the ability to respond to extracellular stimuli and to regulate osteoclastogenesis; GSD cells proliferate under aCD3/CD28 signal reaching similar levels to those observed in control culture and exert their immunomodulatory activity on effector T cells. GSD Treg cells preserved their inhibitory effects on the osteoclastogenesis. These results suggest that stimulation of Treg cells could open the way for the identification and testing of new therapeutic approaches for patients affected by GSD.https://www.frontiersin.org/articles/10.3389/fcell.2021.706596/fulldiseases and disorders of/related to boneosteoclastsosteoimmunologyregulatory T cellsGorham-Stout disease |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Michela Rossi Ippolita Rana Paola Sabrina Buonuomo Giulia Battafarano Viviana De Martino Matteo D’Agostini Ottavia Porzio Ottavia Porzio Cristiana Cipriani Salvatore Minisola Rita De Vito Davide Vecchio Michaela Veronika Gonfiantini Alessandro Jenkner Andrea Bartuli Andrea Del Fattore |
spellingShingle |
Michela Rossi Ippolita Rana Paola Sabrina Buonuomo Giulia Battafarano Viviana De Martino Matteo D’Agostini Ottavia Porzio Ottavia Porzio Cristiana Cipriani Salvatore Minisola Rita De Vito Davide Vecchio Michaela Veronika Gonfiantini Alessandro Jenkner Andrea Bartuli Andrea Del Fattore Stimulation of Treg Cells to Inhibit Osteoclastogenesis in Gorham-Stout Disease Frontiers in Cell and Developmental Biology diseases and disorders of/related to bone osteoclasts osteoimmunology regulatory T cells Gorham-Stout disease |
author_facet |
Michela Rossi Ippolita Rana Paola Sabrina Buonuomo Giulia Battafarano Viviana De Martino Matteo D’Agostini Ottavia Porzio Ottavia Porzio Cristiana Cipriani Salvatore Minisola Rita De Vito Davide Vecchio Michaela Veronika Gonfiantini Alessandro Jenkner Andrea Bartuli Andrea Del Fattore |
author_sort |
Michela Rossi |
title |
Stimulation of Treg Cells to Inhibit Osteoclastogenesis in Gorham-Stout Disease |
title_short |
Stimulation of Treg Cells to Inhibit Osteoclastogenesis in Gorham-Stout Disease |
title_full |
Stimulation of Treg Cells to Inhibit Osteoclastogenesis in Gorham-Stout Disease |
title_fullStr |
Stimulation of Treg Cells to Inhibit Osteoclastogenesis in Gorham-Stout Disease |
title_full_unstemmed |
Stimulation of Treg Cells to Inhibit Osteoclastogenesis in Gorham-Stout Disease |
title_sort |
stimulation of treg cells to inhibit osteoclastogenesis in gorham-stout disease |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Cell and Developmental Biology |
issn |
2296-634X |
publishDate |
2021-08-01 |
description |
Gorham-Stout disease (GSD) is a very rare syndrome displaying excessive bone erosion and vascular lesion. Due to the rarity of the disease and to the limited studies, its etiopathogenesis is not entirely known. The involvement of immune system in the progressive osteolysis was recently suggested. Indeed, extensive reciprocal interactions between the immune and skeletal systems have been demonstrated. This study aimed to evaluate alterations of immune cells in GSD. An increase of CD8+ cells and reduction of CD4+ and CD4+CD25+CD127low cells was revealed in patients. Interestingly, patients’ regulatory T cells maintain the ability to respond to extracellular stimuli and to regulate osteoclastogenesis; GSD cells proliferate under aCD3/CD28 signal reaching similar levels to those observed in control culture and exert their immunomodulatory activity on effector T cells. GSD Treg cells preserved their inhibitory effects on the osteoclastogenesis. These results suggest that stimulation of Treg cells could open the way for the identification and testing of new therapeutic approaches for patients affected by GSD. |
topic |
diseases and disorders of/related to bone osteoclasts osteoimmunology regulatory T cells Gorham-Stout disease |
url |
https://www.frontiersin.org/articles/10.3389/fcell.2021.706596/full |
work_keys_str_mv |
AT michelarossi stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT ippolitarana stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT paolasabrinabuonuomo stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT giuliabattafarano stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT vivianademartino stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT matteodagostini stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT ottaviaporzio stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT ottaviaporzio stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT cristianacipriani stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT salvatoreminisola stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT ritadevito stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT davidevecchio stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT michaelaveronikagonfiantini stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT alessandrojenkner stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT andreabartuli stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease AT andreadelfattore stimulationoftregcellstoinhibitosteoclastogenesisingorhamstoutdisease |
_version_ |
1717817346129657856 |