Role of transforming growth factor-β1 in the pathogenesis of moyamoya disease

mObject. Prominent features of moyamoya disease are intimal thickening of the cerebral arterial trunks and abundant angiogenesis for collateral 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 chain reaction to analyze the expression level of TGF beta(1) in smooth-muscle cells cultured from the superficial temporal arteries (STAs) and measured the serum level of TGF beta(1) by using enzyme-linked immunosorbent assay. Although the STA is not predominantly involved with moyamoya disease, it has been used in studies of the pathogenesis of this disease. In this report, the STAs from six patients with moyamoya disease and four with arteriosclerotic cerebrovascular disease, along with sera from 14 patients with moyamoya disease and 10 normal healthy volunteers, were studied. The expression of TGF beta(1) was significantly higher in cultured smooth-muscle cells derived from the STAs of patients with moyamoya disease than in those derived from the STAs of patients with arteriosclerotic cerebrovascular disease (p < 0.05). The serum level of TGF beta(1) was also significantly higher in patients with 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 disease, including abundant neovascularization, although their findings do not necessarily mean that TGF beta(1) is a causative factor in this disease.