WHSC2, a novel gene recently isolated within the critical region of Wolf–Hirschhorn syndrome, is expressed in endothelial cells. WHSC2 is downregulated by HIV-1 Tat, whereas it is not modulated by angiogenic and pro-differentiative molecules. WHSC2 encodes a basic polypeptide of 528 amino acids. The in vitro translated protein shows a molecular weight of 57 kDa. WHSC2 has two nuclear translocation sequences which actively mediate its transport to the nucleus, as shown in whsc2-GFP-transfected NIH-3T3. We also found a helix-loop-helix (HLH) motif in region 130–185. Since members of the HLH family control differentiation and cell cycle progression, we hypothesize that WHSC2 may function as a transcriptional repressor.
