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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Bibliographische Detailangaben
Veröffentlicht in:Dis Model Mech
Hauptverfasser: 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
Format: Artigo
Sprache:Inglês
Veröffentlicht: The Company of Biologists Ltd 2018
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Resource Article
Online Zugang: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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