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The zinc transporter SLC39A13/ZIP13 is required for connective tissue development : its involvement in BMP/TGF-beta signaling pathways

Fukada, T. and Civic, N. and Furuichi, T. and Shimoda, S. and Mishima, K. and Higashiyama, H. and Idaira, Y. and Asada, Y. and Kitamura, H. and Yamasaki, S. and Hojyo, S. and Nakayama, M. and Ohara, O. and Koseki, H. and Dos Santos, H. G. and Bonafe, L. and Ha-Vinh, R. and Zankl, A. and Unger, S. and Kraenzlin, M. E. and Beckmann, J. S. and Saito, I. and Rivolta, C. and Ikegawa, S. and Superti-Furga, A. and Hirano, T.. (2008) The zinc transporter SLC39A13/ZIP13 is required for connective tissue development : its involvement in BMP/TGF-beta signaling pathways. PLoS one, Vol. 3, H. 11 , e3642.

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Official URL: http://edoc.unibas.ch/dok/A6002821

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Abstract

BACKGROUND: Zinc (Zn) is an essential trace element and it is abundant in connective tissues, however biological roles of Zn and its transporters in those tissues and cells remain unknown. METHODOLOGY/PRINCIPAL FINDINGS: Here we report that mice deficient in Zn transporter Slc39a13/Zip13 show changes in bone, teeth and connective tissue reminiscent of the clinical spectrum of human Ehlers-Danlos syndrome (EDS). The Slc39a13 knockout (Slc39a13-KO) mice show defects in the maturation of osteoblasts, chondrocytes, odontoblasts, and fibroblasts. In the corresponding tissues and cells, impairment in bone morphogenic protein (BMP) and TGF-beta signaling were observed. Homozygosity for a SLC39A13 loss of function mutation was detected in sibs affected by a unique variant of EDS that recapitulates the phenotype observed in Slc39a13-KO mice. CONCLUSIONS/SIGNIFICANCE: Hence, our results reveal a crucial role of SLC39A13/ZIP13 in connective tissue development at least in part due to its involvement in the BMP/TGF-beta signaling pathways. The Slc39a13-KO mouse represents a novel animal model linking zinc metabolism, BMP/TGF-beta signaling and connective tissue dysfunction.
Faculties and Departments:03 Faculty of Medicine > Bereich Medizinische Fächer (Klinik) > Endokrinologie / Diabetologie
03 Faculty of Medicine > Departement Klinische Forschung > Bereich Medizinische Fächer (Klinik) > Endokrinologie / Diabetologie
UniBasel Contributors:Kraenzlin, Marius E.
Item Type:Article, refereed
Article Subtype:Research Article
Bibsysno:Link to catalogue
Publisher:Public Library of Science
ISSN:1932-6203
Note:Publication type according to Uni Basel Research Database: Journal article
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Last Modified:25 Apr 2014 08:01
Deposited On:25 Apr 2014 08:01

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