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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Detalhes bibliográficos
Publicado no:Dis Model Mech
Main Authors: 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: Artigo
Idioma:Inglês
Publicado em: The Company of Biologists Ltd 2018
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Resource Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5963856/
https://ncbi.nlm.nih.gov/pubmed/29666143
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1242/dmm.031542
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