Article

Dysregulated TGFβ-ERK Signaling Drives Aberrant Extracellular Matrix Production in Noonan Syndrome-Associated Pulmonary Valve Stenosis

2026-01-20

Abstract excerpt

Pulmonary valve stenosis (PVS) is the most common congenital heart defect in Noonan syndrome (NS) and related RASopathies, yet the molecular mechanisms linking pathogenic variants to the valve pathology remain poorly defined. Here, we utilized a human iPSC-based valve differentiation platform to generate the cardiac valve cell lineages—including fibrosa and spongiosa valve interstitial cell (VIC) subtypes. CRISPR-...

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Literature Corpus work: 56e49b61-f7dc-510f-9de8-3792ff28d6dd
DOI: 10.64898/2026.01.16.700032

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