IMR Press / FBE / Volume 3 / Issue 3 / DOI: 10.2741/E305

Frontiers in Bioscience-Elite (FBE) is published by IMR Press from Volume 13 Issue 2 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on as a courtesy and upon agreement with Frontiers in Bioscience.

Alterations of elastic fibers in genetically modified mice and human genetic diseases
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1 Inserm UMR 698, Universite Paris 7, Hopital Bichat-Claude Bernard, 46, rue Henri Huchard, 75877, Paris, Cedex 18, France

*Author to whom correspondence should be addressed.

Academic Editor: Maurice Pagano

Front. Biosci. (Elite Ed) 2011, 3(3), 994–1004;
Published: 1 June 2011

The two main components of elastic fibers are elastin and microfibrils. Fibrillin and microfibrillar-associated glycoproteins are the essential constituents of microfibrils. In the last ten years, the analysis of genetically modified mice and human genetic diseases has led to the observation that, besides elastin, fibrillins and microfibrillar-associated proteins, numerous other molecules play an essential role in the supramolecular organisation of the elastic fibers in the extracellular space and in the interactions between elastic fibers and cells. These recent data are summarized in this review.

Preterm Neonates
Non Invasive Analysis
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