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Article

Mutations of extracellular matrix components in vascular disease.

The Annals of thoracic surgery - 1 Jun 1999

Ramirez F, Pereira L

Abstract excerpt

BACKGROUND: Marfan's syndrome (MFS) is characterized by manifestations in the skeletal, ocular, and cardiovascular systems. Dilatation of the aortic root is the hallmark feature in the cardiovascular system. Aortic dilatation is associated with fragmented elastic fibers and accumulation of amorphous matrix elements in the medial layer. This pathology is caused by mutations in fibrillin 1, the major structural...

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Identifiers

PMID: 10391325
DOI: 10.1016/s0003-4975(99)00437-3

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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