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Osteoarthritis-Like Changes in Bardet–Biedl Syndrome Mutant Ciliopathy Mice (Bbs1M390R/M390R): Evidence for a Role of Primary Cilia in Cartilage Homeostasis and Regulation of Inflammation
Journal article   Open access   Peer reviewed

Osteoarthritis-Like Changes in Bardet–Biedl Syndrome Mutant Ciliopathy Mice (Bbs1M390R/M390R): Evidence for a Role of Primary Cilia in Cartilage Homeostasis and Regulation of Inflammation

Isaac D Sheffield, Mercedes A McGee, Steven J Glenn, Da Young Baek, Joshua M Coleman, Bradley K Dorius, Channing Williams, Brandon J Rose, Anthony E Sanchez, Michael A Goodman, …
Frontiers in physiology, Vol.9, pp.708-708
06/19/2018
DOI: 10.3389/fphys.2018.00708
PMCID: PMC6018413
PMID: 29971011
url
https://doi.org/10.3389/fphys.2018.00708View
Published (Version of record) Open Access

Abstract

Osteoarthritis (OA) is a debilitating inflammation related disease characterized by joint pain and effusion, loss of mobility, and deformity that may result in functional joint failure and significant impact on quality of life. Once thought of as a simple “wear and tear” disease, it is now widely recognized that OA has a considerable metabolic component and is related to chronic inflammation. Defects associated with primary cilia have been shown to be cause OA-like changes in Bardet–Biedl mice. We examined the role of dysfunctional primary cilia in OA in mice through the regulation of the previously identified degradative and pro-inflammatory molecular pathways common to OA. We observed an increase in the presence of pro-inflammatory markers TGFβ-1 and HTRA1 as well as cartilage destructive protease MMP-13 but a decrease in DDR-2. We observed a morphological difference in cartilage thickness in Bbs1 M390R/M390R mice compared to wild type (WT). We did not observe any difference in OARSI or Mankin scores between WT and Bbs1 M390R/M390R mice. Primary cilia appear to be involved in the upregulation of biomarkers, including pro-inflammatory markers common to OA.
Bbs Physiology inflammation osteoarthritis primary cilia Bardet–Biedl syndrome

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