2020 Volume 41 Issue 1 Pages 16-22
Marfan syndrome (MFS) is a systemic disorder of connective tissue caused by mutations in the Fibrillin-1 gene (FBN1) that affects the cardiovascular, skeletal and ocular system. Orofacial characteristics are used in the diagnosis of this syndrome, consisting of retrognathic mandible, temporomandibular joint alterations, high arched palate, dental crowding, and posterior crossbite, which have a higher prevalence and severity in patients with MFS. Severe periodontitis is deeply involved in deterioration of aortic condition. For patients with MFS it is important to elucidate the onset mechanisms of periodontitis. Fibrillin-1 encoded by the FBN1 gene consists of periodontal ligament (PDL) and contributes to the formation and maintenance of PDL. Thus, previous studies have focused on PDL in cases of severe periodontitis. There is no report on using gingival fibroblasts, which are a component of periodontal tissue. In addition, few studies have focused on tissue inflammation. Therefore, we examined the use of gingival fibroblasts from monozygotic twins with MFS to clarify the onset of chronic inflammation such as periodontitis in MFS. There were significant differences between twins in the pattern of gene expressions, transforming growth factor-β type I receptor, Interleukin-6, and matrix metalloproteinases. Furthermore, gene expressions in gingival fibroblasts from Marfan syndrome with more serious cardiovascular conditions were higher compared with the other.
