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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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Detalles Bibliográficos
Autores principales: Chen, Mao, Yao, Bing, Yang, Qiangbing, Deng, Jichao, Song, Yuning, Sui, Tingting, Zhou, Lina, Yao, HaoBing, Xu, Yuanyuan, Ouyang, Hongsheng, Pang, Daxin, Li, Zhanjun, Lai, Liangxue
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Ltd 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5963856/
https://www.ncbi.nlm.nih.gov/pubmed/29666143
http://dx.doi.org/10.1242/dmm.031542