Marfan Syndrome and FBN1 Gene Mutations _ Genome Disfunction Review

Marfan Syndrome (MFS) is a systemic connective tissue disorder caused by mutations in the FBN1 gene, which encodes fibrillin-1, a glycoprotein critical to the structural integrity of connective tissues. This autosomal dominant condition primarily affects the cardiovascular, skeletal, and ocular systems. Patients with MFS are at heightened risk for aortic aneurysms and dissections, necessitating vigilant monitoring and early intervention. This article explores the genetic and clinical landscape of Marfan Syndrome and highlights advances in targeted therapies and surgical interventions that aim to improve life expectancy and quality of life.

Publication Details

Journal
Figshare
Published
2024-12-11
DOI
https://doi.org/10.6084/m9.figshare.28009148
Primary Topic
Connective tissue disorders research
Type
article
Field-Weighted Citation Impact
0.00
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article

Marfan Syndrome and FBN1 Gene Mutations _ Genome Disfunction Review

Figshare
Connective tissue disorders research
article

Marfan Syndrome and FBN1 Gene Mutations _ Genome Disfunction Review

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Abstract

Marfan Syndrome (MFS) is a systemic connective tissue disorder caused by mutations in the FBN1 gene, which encodes fibrillin-1, a glycoprotein critical to the structural integrity of connective tissues. This autosomal dominant condition primarily affects the cardiovascular, skeletal, and ocular systems. Patients with MFS are at heightened risk for aortic aneurysms and dissections, necessitating vigilant monitoring and early intervention. This article explores the genetic and clinical landscape of Marfan Syndrome and highlights advances in targeted therapies and surgical interventions that aim to improve life expectancy and quality of life.

Figshare
Openalex Percentile: Top 55%
Connective tissue disorders research
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Marfan Syndrome and FBN1 Gene Mutations _ Genome Disfunction Review · Figshare (2024) | TGRS Research Map | TGRS