Cargando…

GLI1 and AXIN2 Are Distinctive Markers of Human Calvarial Mesenchymal Stromal Cells in Nonsyndromic Craniosynostosis

All skeletal bones house osteogenic stem cell niches, in which mesenchymal stromal cells (MSC) provide progenitors for tissue growth and regeneration. They have been widely studied in long bones formed through endochondral ossification. Limited information is available on the composition of the oste...

Descripción completa

Detalles Bibliográficos
Autores principales: Di Pietro, Lorena, Barba, Marta, Prampolini, Chiara, Ceccariglia, Sabrina, Frassanito, Paolo, Vita, Alessia, Guadagni, Enrico, Bonvissuto, Davide, Massimi, Luca, Tamburrini, Gianpiero, Parolini, Ornella, Lattanzi, Wanda
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7352200/
https://www.ncbi.nlm.nih.gov/pubmed/32575385
http://dx.doi.org/10.3390/ijms21124356
_version_ 1783557582080704512
author Di Pietro, Lorena
Barba, Marta
Prampolini, Chiara
Ceccariglia, Sabrina
Frassanito, Paolo
Vita, Alessia
Guadagni, Enrico
Bonvissuto, Davide
Massimi, Luca
Tamburrini, Gianpiero
Parolini, Ornella
Lattanzi, Wanda
author_facet Di Pietro, Lorena
Barba, Marta
Prampolini, Chiara
Ceccariglia, Sabrina
Frassanito, Paolo
Vita, Alessia
Guadagni, Enrico
Bonvissuto, Davide
Massimi, Luca
Tamburrini, Gianpiero
Parolini, Ornella
Lattanzi, Wanda
author_sort Di Pietro, Lorena
collection PubMed
description All skeletal bones house osteogenic stem cell niches, in which mesenchymal stromal cells (MSC) provide progenitors for tissue growth and regeneration. They have been widely studied in long bones formed through endochondral ossification. Limited information is available on the composition of the osteogenic niche in flat bones (i.e., skull vault bones) that develop through direct membranous ossification. Craniosynostosis (CS) is a congenital craniofacial defect due to the excessive and premature ossification of skull vault sutures. This study aimed at analysing the expression of GLI1, AXIN2 and THY1 in the context of the human skull vault, using nonsyndromic forms of CS (NCS) as a model to test their functional implication in the aberrant osteogenic process. The expression of selected markers was studied in NCS patients’ calvarial bone specimens, to assess the in vivo location of cells, and in MSC isolated thereof. The marker expression profile was analysed during in vitro osteogenic differentiation to validate the functional implication. Our results show that GLI1 and AXIN2 are expressed in periosteal and endosteal locations within the osteogenic niche of human calvarial bones. Their expression is higher in MSC isolated from calvarial bones than in those isolated from long bones and tends to decrease upon osteogenic commitment and differentiation. In particular, AXIN2 expression was lower in cells isolated from prematurely fused sutures than in those derived from patent sutures of NCS patients. This suggests that AXIN2 could reasonably represent a marker for the stem cell population that undergoes depletion during the premature ossification process occurring in CS.
format Online
Article
Text
id pubmed-7352200
institution National Center for Biotechnology Information
language English
publishDate 2020
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-73522002020-07-15 GLI1 and AXIN2 Are Distinctive Markers of Human Calvarial Mesenchymal Stromal Cells in Nonsyndromic Craniosynostosis Di Pietro, Lorena Barba, Marta Prampolini, Chiara Ceccariglia, Sabrina Frassanito, Paolo Vita, Alessia Guadagni, Enrico Bonvissuto, Davide Massimi, Luca Tamburrini, Gianpiero Parolini, Ornella Lattanzi, Wanda Int J Mol Sci Article All skeletal bones house osteogenic stem cell niches, in which mesenchymal stromal cells (MSC) provide progenitors for tissue growth and regeneration. They have been widely studied in long bones formed through endochondral ossification. Limited information is available on the composition of the osteogenic niche in flat bones (i.e., skull vault bones) that develop through direct membranous ossification. Craniosynostosis (CS) is a congenital craniofacial defect due to the excessive and premature ossification of skull vault sutures. This study aimed at analysing the expression of GLI1, AXIN2 and THY1 in the context of the human skull vault, using nonsyndromic forms of CS (NCS) as a model to test their functional implication in the aberrant osteogenic process. The expression of selected markers was studied in NCS patients’ calvarial bone specimens, to assess the in vivo location of cells, and in MSC isolated thereof. The marker expression profile was analysed during in vitro osteogenic differentiation to validate the functional implication. Our results show that GLI1 and AXIN2 are expressed in periosteal and endosteal locations within the osteogenic niche of human calvarial bones. Their expression is higher in MSC isolated from calvarial bones than in those isolated from long bones and tends to decrease upon osteogenic commitment and differentiation. In particular, AXIN2 expression was lower in cells isolated from prematurely fused sutures than in those derived from patent sutures of NCS patients. This suggests that AXIN2 could reasonably represent a marker for the stem cell population that undergoes depletion during the premature ossification process occurring in CS. MDPI 2020-06-19 /pmc/articles/PMC7352200/ /pubmed/32575385 http://dx.doi.org/10.3390/ijms21124356 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Di Pietro, Lorena
Barba, Marta
Prampolini, Chiara
Ceccariglia, Sabrina
Frassanito, Paolo
Vita, Alessia
Guadagni, Enrico
Bonvissuto, Davide
Massimi, Luca
Tamburrini, Gianpiero
Parolini, Ornella
Lattanzi, Wanda
GLI1 and AXIN2 Are Distinctive Markers of Human Calvarial Mesenchymal Stromal Cells in Nonsyndromic Craniosynostosis
title GLI1 and AXIN2 Are Distinctive Markers of Human Calvarial Mesenchymal Stromal Cells in Nonsyndromic Craniosynostosis
title_full GLI1 and AXIN2 Are Distinctive Markers of Human Calvarial Mesenchymal Stromal Cells in Nonsyndromic Craniosynostosis
title_fullStr GLI1 and AXIN2 Are Distinctive Markers of Human Calvarial Mesenchymal Stromal Cells in Nonsyndromic Craniosynostosis
title_full_unstemmed GLI1 and AXIN2 Are Distinctive Markers of Human Calvarial Mesenchymal Stromal Cells in Nonsyndromic Craniosynostosis
title_short GLI1 and AXIN2 Are Distinctive Markers of Human Calvarial Mesenchymal Stromal Cells in Nonsyndromic Craniosynostosis
title_sort gli1 and axin2 are distinctive markers of human calvarial mesenchymal stromal cells in nonsyndromic craniosynostosis
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7352200/
https://www.ncbi.nlm.nih.gov/pubmed/32575385
http://dx.doi.org/10.3390/ijms21124356
work_keys_str_mv AT dipietrolorena gli1andaxin2aredistinctivemarkersofhumancalvarialmesenchymalstromalcellsinnonsyndromiccraniosynostosis
AT barbamarta gli1andaxin2aredistinctivemarkersofhumancalvarialmesenchymalstromalcellsinnonsyndromiccraniosynostosis
AT prampolinichiara gli1andaxin2aredistinctivemarkersofhumancalvarialmesenchymalstromalcellsinnonsyndromiccraniosynostosis
AT ceccarigliasabrina gli1andaxin2aredistinctivemarkersofhumancalvarialmesenchymalstromalcellsinnonsyndromiccraniosynostosis
AT frassanitopaolo gli1andaxin2aredistinctivemarkersofhumancalvarialmesenchymalstromalcellsinnonsyndromiccraniosynostosis
AT vitaalessia gli1andaxin2aredistinctivemarkersofhumancalvarialmesenchymalstromalcellsinnonsyndromiccraniosynostosis
AT guadagnienrico gli1andaxin2aredistinctivemarkersofhumancalvarialmesenchymalstromalcellsinnonsyndromiccraniosynostosis
AT bonvissutodavide gli1andaxin2aredistinctivemarkersofhumancalvarialmesenchymalstromalcellsinnonsyndromiccraniosynostosis
AT massimiluca gli1andaxin2aredistinctivemarkersofhumancalvarialmesenchymalstromalcellsinnonsyndromiccraniosynostosis
AT tamburrinigianpiero gli1andaxin2aredistinctivemarkersofhumancalvarialmesenchymalstromalcellsinnonsyndromiccraniosynostosis
AT paroliniornella gli1andaxin2aredistinctivemarkersofhumancalvarialmesenchymalstromalcellsinnonsyndromiccraniosynostosis
AT lattanziwanda gli1andaxin2aredistinctivemarkersofhumancalvarialmesenchymalstromalcellsinnonsyndromiccraniosynostosis