Truncated C-terminus of fibrillin-1 induces Marfanoid-progeroid-lipodystrophy (MPL) syndrome in rabbit

• Egile Nagusiak: Mao Chen (Egilea), Bing Yao (Egilea), Qiangbing Yang (Egilea), Jichao Deng (Egilea), Yuning Song (Egilea), Tingting Sui (Egilea), Lina Zhou (Egilea), HaoBing Yao (Egilea), Yuanyuan Xu (Egilea), Hongsheng Ouyang (Egilea), Daxin Pang (Egilea), Zhanjun Li (Egilea), Liangxue Lai (Egilea)
• Formatua: Liburua
• Argitaratua: The Company of Biologists, 2018-04-01T00:00:00Z.
• Gaiak: article
• Sarrera elektronikoa: Connect to this object online.

Various clinical differences have been observed between patients with the FBN1 gene mutation and those with the classical Marfan phenotype. Although FBN1 knockout (KO) or dominant-negative mutant mice are widely used as an animal model for Marfan syndrome (MFS), these mice cannot recapitulate the ge...