Genotype-associated structural and nanomechanical alterations of aortic fibrillin-1 microfibrils in Marfan syndrome

Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder caused by mutations in the gene encoding fibrillin-1 (FBN1), the main component of extracellular microfibrils. In the aortic wall, these microfibrils maintain structural integrity and sustain hemodynamic load. Pathogenic FBN1...

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主要な著者:	Cristina M. Șulea, Dominik Sziklai, Bálint Kiss, Kálmán Benke, Miklós Pólos, Bence Ágg, Roland Stengl, Máté Csonka, Zoltán Szabolcs, Miklós S.Z. Kellermayer
フォーマット:	Artigo
言語:	Inglês
出版事項:	Elsevier 2026-12-01
シリーズ:	Matrix Biology Plus
主題:	Marfan syndrome Fibrillin-1 microfibrils Extracellular matrix Inherited connective tissue disorders Cardiovascular disease Atomic force microscopy
オンライン･アクセス:	http://www.sciencedirect.com/science/article/pii/S2590028526000165
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