Neural Crest-Derived Endocardial Cells Required for Heart Valve Development

Neural crest-derived, highly secretory valve endocardial cells expressing Pthlh are essential for mouse heart valve development, and their loss causes perinatal lethality with thickened leaflets, stenosis, and regurgitation.

• Why it matters: Valve defects affect 2-3% of people worldwide and are a leading structural heart disease, yet the specific cell types and signaling interactions that build and maintain valves are unclear.
• What they did: The study identified a rare valve endocardial subset in mouse and human, then ablated it in mice and disrupted Pthlh globally or via neural crest-specific deletion to test effects on maturation and BMP signaling.
• The result: Ablation and Pthlh disruption both produced valve stenosis and lethality, with neural crest-specific loss impairing valvular interstitial cell maturation and disrupting bone morphogenetic protein signaling, implicating this pathway in human valve disease.

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