ROLE OF TRANSFORMING GROWTH FACTOR-BETA(1) IN THE PATHOGENESIS OF MOYAMOYA-DISEASE

mObject. Prominent features of moyamoya disease are intimal thickening of the cerebral arterial trunks and abundant angiogenesis for collate ral blood supplies, but its pathogenesis is still unknown. The aim of this study was to test the possibility that transforming growth factor -beta(1) (TGF beta(1)) may play a role in the pathogenesis of moyamoya disease. Methods. The authors used reverse transcription-polymerase c hain reaction to analyze the expression level of TGF beta(1) in smooth -muscle cells cultured from the superficial temporal arteries (STAs) a nd measured the serum level of TGF beta(1) by using enzyme-linked immu nosorbent assay. Although the STA is not predominantly involved with m oyamoya disease, it has been used in studies of the pathogenesis of th is disease. In this report, the STAs from six patients with moyamoya d isease and four with arteriosclerotic cerebrovascular disease, along w ith sera from 14 patients with moyamoya disease and 10 normal healthy volunteers, were studied. The expression of TGF beta(1) was significan tly higher in cultured smooth-muscle cells derived from the STAs of pa tients with moyamoya disease than in those derived from the STAs of pa tients with arteriosclerotic cerebrovascular disease (p < 0.05). The s erum level of TGF beta(1) was also significantly higher in patients wi th moyamoya disease than in controls (p < 0.0005). Conclusions. Taking into account the functional roles of TGF beta(1) in the expression of connective tissue genes and angiogenesis, these investigators suggest that TGF beta(1) is associated with the pathogenesis of moyamoya dise ase, including abundant neovascularization, although their findings do not necessarily mean that TGF beta(1) is a causative factor in this d isease.