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Patient-Derived Organoids Recapitulate Pathological Intrinsic and Phenotypic Features of Fibrous Dysplasia

ABSTRACT: Patient-Derived Organoids Recapitulate Pathological Intrinsic and Phenotypic Features of Fibrous Dysplasia

PROVIDER: PRJNA1096652 | ENA |

REPOSITORIES: ENA

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			Action	DRS
	SRR28573932_1.fastq.gz	Fastqsanger.gz
	SRR28573932_2.fastq.gz	Fastqsanger.gz
	SRR28573933_1.fastq.gz	Fastqsanger.gz
	SRR28573933_2.fastq.gz	Fastqsanger.gz
	SRR28573934_1.fastq.gz	Fastqsanger.gz

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Patient-Derived Organoids Recapitulate Pathological Intrinsic and Phenotypic Features of Fibrous Dysplasia

Project description:Fibrous dysplasia (FD) is a rare bone disorder characterized by the replacement of normal bone with benign fibro-osseous tissue. Developments in our understanding of the pathophysiology and treatment options are impeded by the lack of suitable research models. In this study, we developed an in vitro organotypic model capable of recapitulating key intrinsic and phenotypic properties of FD. Transcriptomic profiling of individual cells isolated from patient lesional tissues revealed extensive intralesional molecular and cellular heterogeneity, indicative of pathogenic fibrotic processes.

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Project description:Fibrous dysplasia (FD) and cemento-ossifying fibroma (COF) are benign fibrous-osseous lesions of the maxillofacial bones that exhibit numerous similarities in their clinicopathological aspects. However, due to differences in their clinical evolution and treatment, it is essential to diagnose these diseases with accuracy. The differences in the pathogeneses of FD and COF are largely unknown and currently there is no biomarker available for their differential diagnosis. Nonetheless, there are few studies comparing these lesions and predominantly comprised of immunohistochemical investigations of traditional bone biomarkers. Therefore, the present study aimed to quantitatively compare the proteomic and phosphoproteomic profiles of cemento-ossifying fibroma and fibrous dysplasia to shed light onto the mechanisms involved in their molecular pathogenesis and identify potential differential diagnosis biomarkers.

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Transcriptomic signature and pro-osteoclastic secreted factors of abnormal bone marrow stromal cells in fibrous dysplasia (Mouse)

Project description:Transcriptomic signature and pro-osteoclastic secreted factors of abnormal bone marrow stromal cells in fibrous dysplasia (Mouse)

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Transcriptomic signature and pro-osteoclastic secreted factors of abnormal bone marrow stromal cells in fibrous dysplasia (Mouse)

Project description:Fibrous dysplasia (FD) is a mosaic skeletal disorder caused by somatic activating variants in GNAS, encoding for Gαs, which leads to excessive cAMP signaling in bone marrow stromal cells (BMSCs). Despite advancements in our understanding of FD pathophysiology, the effect of Gαs activation in the BMSC transcriptome remains unclear, as well as how this translates into their local influence in the lesional microenvironment. In this study, we analyzed changes induced by Gαs activation in BMSC transcriptome and performed a comprehensive analysis of their production of cytokines and other secreted factors. We performed RNAseq of cultured BMSCs from patients with FD and healthy volunteers, and from an inducible mouse model of FD, and combined their transcriptomic profiles to build a robust FD BMSC genetic signature. Pathways related to Gαs activation, cytokine signaling, and extracellular matrix deposition were identified. In addition, a comprehensive profile of their secreted cytokines and other factors was performed to identify modulation of several key factors we hypothesized to be involved in FD pathogenesis. We also screened circulating cytokines in a collection of plasma samples from patients with FD, finding positive correlations of several cytokines to their disease burden score, as well as to one another and bone turnover markers. Overall, these data support a pro-inflammatory, pro-osteoclastic behavior of BMSCs bearing hyperactive Gαs variants, and point to several cytokines and other secreted factors as possible therapeutic targets and/or circulating biomarkers for FD.

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