Expression of HES and HEY genes in infantile hemangiomas

摘要: Infantile hemangiomas (IHs) are the most common benign tumor of infancy, yet their pathogenesis is poorly understood. IHs believed to originate from a progenitor cell, hemangioma stem cell (HemSC). Recent studies by our group showed that NOTCH proteins and ligands expressed in hemangiomas, indicating Notch signaling may be active IHs. We sought investigate downstream activation cells evaluating expression basic HLH family proteins, HES/HEY, HemSCs endothelial (HemECs) isolated freshly resected specimens. Quantitative RT-PCR was performed probe for relative gene transcript levels (normalized beta-actin). Immunofluorescence evaluate protein expression. Co-localization were with CD31 (endothelial cells) NOTCH3 (peri-vascular, non-endothelial cells). treated gamma secretase inhibitor (GSI) Compound E, quantified real-time PCR. HEY1, HEYL, HES1 highly HemSCs, while HEY2 HemECs. Protein evaluation immunofluorescence confirms HemECs (CD31+ cells), more widely mostly perivascular hemangiomas. Inhibition addition GSI resulted down-regulation HES/HEY genes. genes type specific patterns; HemSCs. This pattern suggests HEY/HES act receptors function distinct types transcripts decreased gamma-secretase inhibitor, demonstrating infantile cells.