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Atrial ERK1/2 activation in the embryo leads to incomplete Septal closure: a novel mouse model of atrial Septal defect.

ABSTRACT: BACKGROUND:MEK1 mutation and activated MAPK signaling has been found in patients with RASopathies and abnormal cardiac development. Previous studies have suggested that regulation of fetal MAPK signaling is essential for normal cardiac development. We investigated the effect of active MEK1 overexpression on fetal atrial septal development. METHODS AND RESULTS:An inducible double transgenic (DTg) mouse model was developed in which cardiac-specific fetal expression of a constitutively active form of human MEK1 (aMEK1) was induced primarily in the atrium via the withdrawal of doxycycline from the drinking water of pregnant mice. Atrial septal defect (ASD) was found in 51% (23/45) of DTg mice. Fifty-two percent (12/23) of ASD mice died before weaning, and surviving ASD mice exhibited hypertrophic hearts with enlarged right atria and decreased fractional shorting (40?±?2% vs. 48?±?0%, p?

SUBMITTER: Yeh CC

PROVIDER: S-EPMC5702213 | biostudies-literature | 2017 Nov

REPOSITORIES: biostudies-literature

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Atrial ERK1/2 activation in the embryo leads to incomplete Septal closure: a novel mouse model of atrial Septal defect.

Yeh Che-Chung CC Fan Yanying Y Yang Yi-Lin YL Mann Michael J MJ

Journal of biomedical science 20171124 1

<h4>Background</h4>MEK1 mutation and activated MAPK signaling has been found in patients with RASopathies and abnormal cardiac development. Previous studies have suggested that regulation of fetal MAPK signaling is essential for normal cardiac development. We investigated the effect of active MEK1 overexpression on fetal atrial septal development.<h4>Methods and results</h4>An inducible double transgenic (DTg) mouse model was developed in which cardiac-specific fetal expression of a constitutive ...[more]

PMID: 29178881

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