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Title: Tropoelastin regulates chemokine expression in fibroblasts in Costello syndrome

Abstract

Costello syndrome is a multiple congenital anomaly associated with growth and mental retardation, cardiac and skeletal anomalies, and a predisposition to develop neoplasia. Comprehensive expression analysis revealed remarkable up-regulation of several cytokines and chemokines including Gro family proteins, interleukin-1{beta} (IL-1{beta}), IL-8 and MCP-1 but down-regulation of extracellular matrix components including collagens and proteoglycans of skin fibroblasts derived from a Japanese Costello syndrome patient characterized by significantly reduced tropoelastin mRNA, impaired elastogenesis and enhanced cell proliferation. In contrast, decreases in these chemokines and IL-1{beta} expression were observed in Costello fibroblastic cell lines stably expressing the bovine tropoelastin (btEln) gene and in restored elastic fibers. These results strongly suggest that the human TE gene (ELN) transfer could be applicable for the gene therapy of a group of Costello syndrome patients with reduced ELN gene expression.

Authors:
 [1];  [2];  [2];  [3];  [1]; ;  [1];  [3];  [4];  [1]
  1. Department of Medicinal Biotechnology, Institute for Medicinal Resources, Graduate School of Pharmaceutical Sciences, University of Tokushima, 1-78 Sho-machi, Tokushima 770-8505 (Japan)
  2. Glyco-Chain Expression Laboratory, Supra-Biomolecular System Research Group, RIKEN Frontier Research System, Saitama 351-0198 (Japan)
  3. Department of Pediatrics, Akita University of Medical School, Akita 010-8543 (Japan)
  4. Department of Analytical Biochemistry, Meiji Pharmaceutical University, Tokyo 204-8588 (Japan)
Publication Date:
OSTI Identifier:
21143799
Resource Type:
Journal Article
Journal Name:
Biochemical and Biophysical Research Communications
Additional Journal Information:
Journal Volume: 372; Journal Issue: 4; Other Information: DOI: 10.1016/j.bbrc.2008.05.131; PII: S0006-291X(08)01017-6; Copyright (c) 2008 Elsevier Science B.V., Amsterdam, The Netherlands, All rights reserved; Country of input: International Atomic Energy Agency (IAEA); Journal ID: ISSN 0006-291X
Country of Publication:
United States
Language:
English
Subject:
60 APPLIED LIFE SCIENCES; CATTLE; CELL PROLIFERATION; COLLAGEN; DNA; FIBERS; FIBROBLASTS; GENE REGULATION; GENE THERAPY; LYMPHOKINES; NERVOUS SYSTEM DISEASES; PATIENTS; SKIN

Citation Formats

Tatano, Yutaka, CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075, Fujinawa, Reiko, Kozutsumi, Yasunori, Laboratory of Membrane Biochemistry and Biophysics, Graduate School of Biostudy, Faculty of Pharmaceutical Sciences, Kyoto University, Kyoto 606-8501, Takahashi, Tsutomu, Tsuji, Daisuke, CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075, Takeuchi, Naohiro, Tsuta, Kohji, Takada, Goro, Sakuraba, Hitoshi, CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075, Itoh, Kohji, and CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075. Tropoelastin regulates chemokine expression in fibroblasts in Costello syndrome. United States: N. p., 2008. Web. doi:10.1016/j.bbrc.2008.05.131.
Tatano, Yutaka, CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075, Fujinawa, Reiko, Kozutsumi, Yasunori, Laboratory of Membrane Biochemistry and Biophysics, Graduate School of Biostudy, Faculty of Pharmaceutical Sciences, Kyoto University, Kyoto 606-8501, Takahashi, Tsutomu, Tsuji, Daisuke, CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075, Takeuchi, Naohiro, Tsuta, Kohji, Takada, Goro, Sakuraba, Hitoshi, CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075, Itoh, Kohji, & CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075. Tropoelastin regulates chemokine expression in fibroblasts in Costello syndrome. United States. https://doi.org/10.1016/j.bbrc.2008.05.131
Tatano, Yutaka, CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075, Fujinawa, Reiko, Kozutsumi, Yasunori, Laboratory of Membrane Biochemistry and Biophysics, Graduate School of Biostudy, Faculty of Pharmaceutical Sciences, Kyoto University, Kyoto 606-8501, Takahashi, Tsutomu, Tsuji, Daisuke, CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075, Takeuchi, Naohiro, Tsuta, Kohji, Takada, Goro, Sakuraba, Hitoshi, CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075, Itoh, Kohji, and CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075. 2008. "Tropoelastin regulates chemokine expression in fibroblasts in Costello syndrome". United States. https://doi.org/10.1016/j.bbrc.2008.05.131.
@article{osti_21143799,
title = {Tropoelastin regulates chemokine expression in fibroblasts in Costello syndrome},
author = {Tatano, Yutaka and CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075 and Fujinawa, Reiko and Kozutsumi, Yasunori and Laboratory of Membrane Biochemistry and Biophysics, Graduate School of Biostudy, Faculty of Pharmaceutical Sciences, Kyoto University, Kyoto 606-8501 and Takahashi, Tsutomu and Tsuji, Daisuke and CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075 and Takeuchi, Naohiro and Tsuta, Kohji and Takada, Goro and Sakuraba, Hitoshi and CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075 and Itoh, Kohji and CREST, JST, 5, Sanban-cho, Chiyoda-ku, Tokyo 102-0075},
abstractNote = {Costello syndrome is a multiple congenital anomaly associated with growth and mental retardation, cardiac and skeletal anomalies, and a predisposition to develop neoplasia. Comprehensive expression analysis revealed remarkable up-regulation of several cytokines and chemokines including Gro family proteins, interleukin-1{beta} (IL-1{beta}), IL-8 and MCP-1 but down-regulation of extracellular matrix components including collagens and proteoglycans of skin fibroblasts derived from a Japanese Costello syndrome patient characterized by significantly reduced tropoelastin mRNA, impaired elastogenesis and enhanced cell proliferation. In contrast, decreases in these chemokines and IL-1{beta} expression were observed in Costello fibroblastic cell lines stably expressing the bovine tropoelastin (btEln) gene and in restored elastic fibers. These results strongly suggest that the human TE gene (ELN) transfer could be applicable for the gene therapy of a group of Costello syndrome patients with reduced ELN gene expression.},
doi = {10.1016/j.bbrc.2008.05.131},
url = {https://www.osti.gov/biblio/21143799}, journal = {Biochemical and Biophysical Research Communications},
issn = {0006-291X},
number = 4,
volume = 372,
place = {United States},
year = {Fri Aug 08 00:00:00 EDT 2008},
month = {Fri Aug 08 00:00:00 EDT 2008}
}