An endothelial-specific TIE2-L914F mouse model reveals cellular mechanisms of venous malformation progression and recapitulates the patient's response to targeted therapy

Postnatal endothelial expression of TIE2 p.L914F in mice produces venous malformation lesions with dilated veins and irregular smooth muscle coverage, recapitulating patient VM histology.

• Why it matters: Understanding how TIE2-driven VM progresses has been limited by the absence of an in vivo genetic model that can reveal cellular mechanisms and support therapy testing.
• What they did: The authors engineered mice with postnatal endothelial TIE2L914F expression and used an mCherry reporter to track lesion formation, then quantified clonal expansion via an early proliferation burst followed by increased cell size.
• The result: Rapamycin targeted treatment partially reduces lesion growth by affecting proliferation and prolongs mouse survival, indicating this model recapitulates the patients’ therapeutic response.

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