Hereditary haemorrhagic telangiectasia, inherited as an autosomal dominant trait, affects
approximately 1 in 5000 people. The abnormal vascular structures in HHT result from
mutations in genes (most commonly endoglin or ACVRL1) whose protein products influence
TGF-ß superfamily signalling in vascular endothelial cells. The cellular mechanisms
underlying the generation of HHT telangiectasia and arteriovenous malformations are being
unravelled, with recent data focussing on a defective response to angiogenic stimuli in …