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Article

The Tight skin mouse: demonstration of mutant fibrillin-1 production and assembly into abnormal microfibrils.

The Journal of cell biology - 9 Mar 1998

Kielty C M, Raghunath M, Siracusa L D, Sherratt M J, Peters R, Shuttleworth C A, Jimenez S A

Abstract excerpt

Mice carrying the Tight skin (Tsk) mutation harbor a genomic duplication within the fibrillin-1 (Fbn 1) gene that results in a larger than normal in-frame Fbn 1 transcript. In this study, the consequences of the Tsk mutation for fibrillin-containing microfibrils have been examined. Dermal fibroblasts from Tsk/+ mice synthesized and secreted both normal fibrillin (approximately 330 kD) and the mutant oversized Tsk...

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Identifiers

PMID: 9490728
DOI: 10.1083/jcb.140.5.1159
PMCID: PMC2132699

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

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