Decreased mitochondrial respiration in aneurysmal aortas of Fibulin-4 mutant mice is linked to PGC1A regulation.

doi:10.1093/cvr/cvy150

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Article

Decreased mitochondrial respiration in aneurysmal aortas of Fibulin-4 mutant mice is linked to PGC1A regulation.

Cardiovascular research - 1 Nov 2018

van der Pluijm Ingrid, Burger Joyce, van Heijningen Paula M, IJpma Arne, van Vliet Nicole, Milanese Chiara, Schoonderwoerd Kees, Sluiter Willem, Ringuette Lea-Jeanne, Dekkers Dirk H W, Que Ivo, Kaijzel Erik L, Te Riet Luuk, MacFarlane Elena G, Das Devashish, van der Linden Reinier, Vermeij Marcel, Demmers Jeroen A, Mastroberardino Pier G, Davis Elaine C, Yanagisawa Hiromi, Dietz Harry C, Kanaar Roland, Essers Jeroen

Abstract excerpt

Aim: Thoracic aortic aneurysms are a life-threatening condition often diagnosed too late. To discover novel robust biomarkers, we aimed to better understand the molecular mechanisms underlying aneurysm formation. Methods and results: In Fibulin-4R/R mice, the extracellular matrix protein Fibulin-4 is 4-fold reduced, resulting in progressive ascending aneurysm formation and early death around 3 months of age. We...

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Identifiers

PMID: 29931197
DOI: 10.1093/cvr/cvy150
PMCID: PMC6198735

Genes

FBN1
SMAD3
TGFBR2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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