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

Experimental cell research - 15 May 2006

Menon Rajesh P, Menon Malini R, Shi-Wen Xu, Renzoni Elisabetta, Bou-Gharios George, Black Carol M, Abraham David J

Abstract excerpt

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 and spontaneously develop connective tissue disease. In this study, we generated hammerhead ribozymes directed...

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Identifiers

PMID: 16488411
DOI: 10.1016/j.yexcr.2006.01.011

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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