Saito Masahiro, Tsuji Takashi
Department of Biological Science and Technology, Tokyo University of Science, Japan.
Clin Calcium. 2012 Jan;22(1):35-42.
Marfan's syndrome (MFS) is a systemic disorder of the connective tissues caused by insufficient fibrillin-1 microfibril formation and can cause cardiac complications, emphysema, ocular lens dislocation and severe periodontal disease. ADAMTSL6β, a microfibril-associated extracellular matrix protein that has been implicated in fibrillin-1 microfibril assembly is able to improve microfibril insufficiency in MFS mice model. These findings suggest a new therapeutic strategy for the treatment of MFS through ADAMTSL6β-mediated fibrillin-1 microfibril assembly. We here review effect on ADAMTSL6β to the improvement of microfibril insufficiency in periodontal tissue as a model.
马凡综合征(MFS)是一种由原纤蛋白-1微原纤维形成不足引起的结缔组织全身性疾病,可导致心脏并发症、肺气肿、晶状体脱位和严重的牙周病。ADAMTSL6β是一种与微原纤维相关的细胞外基质蛋白,参与原纤蛋白-1微原纤维组装,能够改善马凡综合征小鼠模型中的微原纤维不足。这些发现提示了一种通过ADAMTSL6β介导的原纤蛋白-1微原纤维组装来治疗马凡综合征的新治疗策略。我们在此综述以牙周组织为模型时ADAMTSL6β对改善微原纤维不足的作用。