Characterization of the Zebrafish () Mutant: A New Model of Elastinopathy Leading to Heart Valve Defects.

Cells

Laboratoire de Biologie Tissulaire et Ingénierie Thérapeutique (LBTI), UMR CNRS 5305, Institut de Biologie et Chimie des Protéines, Université de Lyon 1, 7 Passage du Vercors, CEDEX 07, F-69367 Lyon, France.

Published: May 2023

Elastic fibers are extracellular macromolecules that provide resilience and elastic recoil to elastic tissues and organs in vertebrates. They are composed of an elastin core surrounded by a mantle of fibrillin-rich microfibrils and are essentially produced during a relatively short period around birth in mammals. Thus, elastic fibers have to resist many physical, chemical, and enzymatic constraints occurring throughout their lives, and their high stability can be attributed to the elastin protein. Various pathologies, called elastinopathies, are linked to an elastin deficiency, such as non-syndromic supravalvular aortic stenosis (SVAS), Williams-Beuren syndrome (WBS), and autosomal dominant cutis laxa (ADCL). To understand these diseases, as well as the aging process related to elastic fiber degradation, and to test potential therapeutic molecules in order to compensate for elastin impairments, different animal models have been proposed. Considering the many advantages of using zebrafish, we here characterize a zebrafish mutant for the paralog () with a specific focus on the cardiovascular system and highlight premature heart valve defects at the adult stage.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10217568PMC
http://dx.doi.org/10.3390/cells12101436DOI Listing

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