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EMILIN-1 Deficiency Induces Elastogenesis and Vascular Cell Defects
Open accessAbstract
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.
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Cited by
Genetic compensation induced by deleterious mutations but not gene knockdowns
Rossi, A., Kontarakis, Z., Gerri, C., Nolte, H., Hölper, S., Krüger, M., & Stainier, D. Y. R. (2015) · Nature · doi:10.1038/nature14580 · Cited by 1,248
Extracellular Matrix Molecules: Potential Targets in Pharmacotherapy
Järveläinen, H., Sainio, A., Koulu, M., Wight, T. N., & Penttinen, R. (2009) · Pharmacological Reviews · doi:10.1124/pr.109.001289 · Cited by 566
New insights into elastic fiber assembly
Wagenseil, J. E., & Mecham, R. P. (2007) · Birth Defects Research Part C: Embryo Today: Reviews · doi:10.1002/bdrc.20111 · Cited by 420
Kanta, J. (2016) · Frontiers in Physiology · doi:10.3389/fphys.2016.00491 · Cited by 316
Hirai, M., Ohbayashi, T., Horiguchi, M., Okawa, K., Hagiwara, A., Chien, K. R., Kita, T., & Nakamura, T. (2007) · The Journal of Cell Biology · doi:10.1083/jcb.200611026 · Cited by 165
Metadata
- Authors
- Zanetti, M., Braghetta, P., Sabatelli, P., Mura, I., Doliana, R., Colombatti, A., Volpin, D., Bonaldo, P., & Bressan, G. M.
- Venue
- Molecular and Cellular Biology
- Publisher
- Informa UK Limited
- Published
- 2004-01-01
- Volume
- 24
- Issue
- 2
- Pages
- 638-650
- ISSN
- 0270-7306 (ISSN-L)
- 1098-5549
- OpenAlex
- W1970559524
- Cited by
- 205
- References
- 0
- FWCI
- 4.02
- Type
- journal article
- Language
- en
- Open access
- green
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- Zanetti, M., Braghetta, P., Sabatelli, P., Mura, I., Doliana, R., Colombatti, A., Volpin, D., Bonaldo, P., & Bressan, G. M. (2004). EMILIN-1 Deficiency Induces Elastogenesis and Vascular Cell Defects. Molecular and Cellular Biology. https://doi.org/10.1128/mcb.24.2.638-650.2004
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