Effect of targeted mutation in collagen V α2 gene on development of cutaneous hyperplasia in tight skin mice

Robert G. Phelps, Chihiro Murai, Shinichiro Saito, Akira Hatakeyama, Konstantinos Andrikopoulos, Kuppuswamy N. Kasturi, Constantin A. Bona

    Research output: Contribution to journalArticlepeer-review

    8 Citations (Scopus)

    Abstract

    Collagen V plays a major regulatory role in the formation of heterotypic fibers of the dermis and cartilaginous tissues as well as in the assembly of extracellular matrix. The pN/pN mouse, which is defective in collagen V α2 gene, exhibits skeletal abnormalities, skin fragility, and alterations in the collagen fiber organization, whereas the TSK/+ mouse, which is defective in fibrillin-1, the major component of microfibrils present in the extracellular matrix, develops cutaneous hyperplasia and autoimmunity. We have studied the role of collagen V in the formation of heterotypic collagen fibers in F1 mice, which are obtained by breeding pN/pN with TSK/+ mice. Our results show that F1 progeny neither develop cutaneous hyperplasia nor produce anti- topoisomerase I autoantibodies, unlike TSK/+ mice. The diameter of the collagen fibrils in the skin is also comparable to that found in control mice. Thus, the phenotypic changes observed in the TSK mouse could be reversed by genetic complementation with a collagen V-defective mouse.

    Original languageEnglish
    Pages (from-to)356-360
    Number of pages5
    JournalMolecular Medicine
    Volume4
    Issue number5
    DOIs
    Publication statusPublished - 1998

    ASJC Scopus subject areas

    • Molecular Medicine
    • Molecular Biology
    • Genetics
    • Genetics(clinical)

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