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

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

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...

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Bibliographic Details
Main Authors: Mao Chen, Bing Yao, Qiangbing Yang, Jichao Deng, Yuning Song, Tingting Sui, Lina Zhou, HaoBing Yao, Yuanyuan Xu, Hongsheng Ouyang, Daxin Pang, Zhanjun Li, Liangxue Lai
Format: Article
Language:English
Published: The Company of Biologists 2018-04-01
Series:Disease Models & Mechanisms
Subjects:
Fibrillin-1
CRISPR/Cas9
Marfanoid-progeroid-lipodystrophy (MPL) syndrome
Rabbit
Online Access:http://dmm.biologists.org/content/11/4/dmm031542

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