Hammerhead ribozyme-mediated silencing of the mutant fibrillin-1 of tight skin mouse: insight into the functional role of mutant fibrillin-1.

The tight skin (Tsk/+) mouse is a model for fibrotic disorders. The genetic defect in the Tsk/+ is an in-frame duplication between exons 17 and 40 of the fibrillin-1 gene which gives rise to a large transcript and protein. Mice homozygous for the mutation die in utero, whereas heterozygotes survive...

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Bibliographic Details
Main Authors: Menon, R, Menon, MR, Shi-Wen, X, Renzoni, E, Bou-Gharios, G, Black, C, Abraham, D
Format: Journal article
Language:English
Published: 2006