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Molecular and Cellular Biology, January 2004, p. 638-650, Vol. 24, No. 2
0270-7306/04/$08.00+0     DOI: 10.1128/MCB.24.2.638-650.2004
Copyright © 2004, American Society for Microbiology. All Rights Reserved.

EMILIN-1 Deficiency Induces Elastogenesis and Vascular Cell Defects

Miriam Zanetti,1,{dagger} Paola Braghetta,1,{dagger} Patrizia Sabatelli,2 Isabella Mura,3 Roberto Doliana,4 Alfonso Colombatti,4 Dino Volpin,1 Paolo Bonaldo,1 and Giorgio M. Bressan1*

Department of Histology Microbiology and Medical Biotechnologies, University of Padua, 35100 Padua,1 Istituto per i Trapianti d'Organo e l'Immunocitologia, CNR,2 Unità Neuromuscolare, Istituti Ortopedici Rizzoli, 40136 Bologna,3 Divisione di Oncologia Sperimentale, CRO-IRCCS, 33081 Aviano, Italy4

Received 26 March 2003/ Returned for modification 28 May 2003/ Accepted 8 October 2003

EMILINs constitute a family of genes of the extracellular matrix with high structural similarity. Four genes have been identified so far in human and mouse. To gain insight into the function of this gene family, EMILIN-1 has been inactivated in the mouse by gene targeting. The homozygous animals were fertile and did not show obvious abnormalities. However, histological and ultrastructural examination revealed alterations of elastic fibers in aorta and skin. Formation of elastic fibers by mutant embryonic fibroblasts in culture was also abnormal. Additional alterations were observed in cell morphology and anchorage of endothelial and smooth muscle cells to elastic lamellae. Considering that EMILIN-1 is adhesive for cells and that the protein binds to elastin and fibulin-5, EMILIN-1 may regulate elastogenesis and vascular cell maintenance by stabilizing molecular interactions between elastic fiber components and by endowing elastic fibers with specific cell adhesion properties.

* Corresponding author. Mailing address: Dipartimento di Istologia Microbiologia e Biotecnologie Mediche, Università di Padova, Viale G. Colombo, 3, 35121 Padua, Italy. Phone: 390498276086. Fax: 390498276079. E-mail: bressan{at}civ.bio.unipd.it.

{dagger} M.Z. and P.B. contributed equally to this work.

Molecular and Cellular Biology, January 2004, p. 638-650, Vol. 24, No. 2
0022-538X/04/$08.00+0     DOI: 10.1128/MCB.24.2.638-650.2004
Copyright © 2004, American Society for Microbiology. All Rights Reserved.



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