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

Journal of Autoimmunity
K N KasturiC Bona

Abstract

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 extracellular matrix. In this study we report the identification of a genetic marker in the Fbn-1 gene that can distinguish the mutant phenotype. TSK mice exhibit an unique polymorphism in the Fbn-1 gene. RNA analysis, PCR analysis and sequence determination of the mutant gene showed that the Fbn-1 gene polymorphism is due to intragenic duplication of a segment of the gene coding for 3.0 Kb of mRNA sequence (10 Kb of the genome). Histological analysis of skin samples from F1 progeny obtained by crossing TSK mice with JH-/-, RAG2-/- or vit/vit showed a significant correlation between the inheritance of the defective Fbn-1 gene and the development of cutaneous hyperplasia. Further, our results also show that in mice deficient in mature B cells inheriting the defective Fbn-1 gene, development of cutaneous hyperplasia is not abrogate...Continue Reading

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Citations

Jun 5, 2002·The Journal of Clinical Investigation·Eriko SaitoShinichi Sato
Nov 9, 2005·The Journal of Clinical Investigation·Rashpal K Bhogal, Constantin A Bona
May 3, 2006·Nihon Rinshō Men'eki Gakkai kaishi = Japanese journal of clinical immunology·Shinichi Sato
Mar 14, 2002·Proceedings of the National Academy of Sciences of the United States of America·Takao KoderaConstantin A Bona
Oct 16, 2004·Molecular Immunology·Shinichi SatoThomas F Tedder
Jan 29, 2008·Seminars in Arthritis and Rheumatism·Y Stephanie GuM Eric Gershwin
Mar 12, 2005·Current Rheumatology Reports·Stephen H Clark
Aug 29, 2006·The American Journal of Pathology·Minoru HasegawaThomas F Tedder
Mar 25, 2006·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Andrew P HoltDavid H Adams
Apr 5, 1997·Annals of the New York Academy of Sciences·K KasturiC Bona
Nov 1, 2002·Current Opinion in Rheumatology·Sergio A Jimenez, Paul J Christner
Nov 25, 2000·Current Opinion in Rheumatology·F K Tan, F C Arnett
Mar 27, 2001·Arthritis and Rheumatism·T D DodigS H Clark
Jun 21, 2007·The Journal of Immunology : Official Journal of the American Association of Immunologists·Yukiyo MatsushitaShinichi Sato
Jul 5, 2003·Rheumatic Diseases Clinics of North America·Filemon K Tan
Jun 22, 2007·The Journal of Investigative Dermatology·Takashi MatsushitaShinichi Sato

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