Projet

1. Identify the functional residues/domain(s) in MGP and examine their roles in Keutel Syndrome- associated cartilage pathologies (MM & HK).

1A. Analyses of the phenotype of Mgp-/- mice expressing Glamut-Mgp in the cartilaginous tissues

1B. Investigate the effects of GGCX deficiency in the cartilaginous tissues. 

1C. Analyses of the cartilage phenotypes of MgpSermut+/- mice

2. Investigate the role of elastin in cartilage calcification caused by MGP deficiency (MM, LC & MC)

2A. Investigate whether elastin content is a critical determinant for cartilage calcification in a zebrafish model.

2B. Determine whether MGP interacts with tropoelastin to prevent elastin mineralization

3. Examine whether the local restoration of Mgp expression in Mgp-/- nasal septum and tracheal chondrocytes prevents ectopic calcification and associated anomalies (MC, MM & HK)

3A. Functionality of the viral vector.

3B. Restoration of Mgp expression in the chondrocytes of the septal cartilage

3C. Restoration of Mgp expresion in the chondrocytes of the tracheal cartilage