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Article

B-cell deficiency does not abrogate development of cutaneous hyperplasia in mice inheriting the defective fibrillin-1 gene.

Journal of autoimmunity - 1 Dec 1997

Kasturi K N, Hatakeyama A, Murai C, Gordon R, Phelps R G, Bona C A

Abstract excerpt

Tight-skin (TSK) mouse, the experimental model for scleroderma, develops cutaneous hyperplasia, cardiac hypertrophy, pulmonary emphysema and autoimmunity against scleroderma target autoantigens. The cutaneous hyperplasia is associated with the accumulation of microfibrils and elastic fibers in the middle and deep dermis. Fibrillin-1 (Fbn-1) is a major component of the 10-12 nm microfibrils found in the...

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Source

Identifiers

PMID: 9451590
DOI: 10.1006/jaut.1997.0158

Genes

RAG2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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B-cell deficiency does not abrogate development of cutaneous hyperplasia in mice inheriting the defective fibrillin-1 gene.PMID 9451590Read ↗

Analysis of the tight skin (Tsk1/+) mouse as a model for testing antifibrotic agents.Semantic proximity Read ↗

Effect of targeted mutation in collagen V alpha 2 gene on development of cutaneous hyperplasia in tight skin mice.Semantic proximity Read ↗

Characterization of mutated protein encoded by partially duplicated fibrillin-1 gene in tight skin (TSK) mice.Semantic proximity Read ↗

Induction of skin fibrosis in mice expressing a mutated fibrillin-1 gene.Semantic proximity Read ↗

New insights into the assembly of extracellular microfibrils from the analysis of the fibrillin 1 mutation in the tight skin mouse.Semantic proximity Read ↗

Mutant fibrillin 1 from tight skin mice increases extracellular matrix incorporation of microfibril-associated glycoprotein 2 and type I collagen.Semantic proximity Read ↗

Structure of the mutant fibrillin-1 gene in the tight skin (TSK) mouse.Semantic proximity Read ↗

Marfan-like skeletal phenotype in the tight skin (Tsk) mouse.Semantic proximity Read ↗

Fibrillin in Marfan syndrome and tight skin mice provides new insights into transforming growth factor-beta regulation and systemic sclerosis.Semantic proximity Read ↗

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

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

A tandem duplication within the fibrillin 1 gene is associated with the mouse tight skin mutation.Semantic proximity Read ↗

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