A role for Sox18 transcription factor has been suggested by lymphatic dysfunction in the human syndrome hypotrichosis-lymphedema-telangiectasia (HLT), which is caused by mutations in Sox18. This paper shows that Sox18 directly activates Prox1 transcription. Sox18-null embryos show a complete absence of Prox1-positive lymphatic endothelial cells emanating from the cardinal vein.
- Mathias François
- Andrea Caprini
- Peter Koopman