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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.

Abstract:

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 against the mutant fibrillin-1 transcript. A partially mispairing ribozyme was the mos...

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Publication status:
Published

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Publisher copy:
10.1016/j.yexcr.2006.01.011

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Institution:
University of Oxford
Department:
Oxford, MSD, NDORMS
Role:
Author
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Journal:
Experimental cell research
Volume:
312
Issue:
9
Pages:
1463-1474
Publication date:
2006-05-05
DOI:
EISSN:
1090-2422
ISSN:
0014-4827
URN:
uuid:a90dba26-7629-412d-a360-0d75a214e52b
Source identifiers:
223507
Local pid:
pubs:223507

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