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

Abstract

Object. 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—β1 (TGFβ1) 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 TGFβ1 in smooth-muscle cells cultured from the superficial temporal arteries (STAs) and measured the serum level of TGFβ1 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 TGFβ1 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 TGFβ1 was also significantly higher in patients with moyamoya disease than in controls (p < 0.0005). Conclusions. Taking into account the functional roles of TGFβ1 in the expression of connective tissue genes and angiogenesis, these investigators suggest that TGFβ1 is associated with the pathogenesis of moyamoya disease, including abundant neovascularization, although their findings do not necessarily mean that TGFβ1 is a causative factor in this disease.

A novel susceptibility locus for moyamoya disease on chromosome 8q23

“…Prominent features of moyamoya disease are intimal thickening of the cerebral arterial trunks and abundant angiogenesis for collateral blood supplies. The expression of transforming growth factor-beta1 (TGFb1) in cultured smooth-muscle cells derived from the superficial temporal arteries and the levels of TGFb1 in serum of patients with moyamoya disease are significantly higher than those of controls (Hojo et al 1998). Therefore, the gene encoding TIEG, transforming growth factor-beta-inducible early growth response, located in 8q22.3, is one of the candidates for moyamoya disease.…”

Increased Levels of Circulating Endothelial Progenitor Cells in Patients With Moyamoya Disease

“…However, unlike tumor angiogenesis, the role of VEGF may be minimal, as evidenced by variable levels of VEGF in the cerebrospinal fluid. [32][33][34][35][36][37] In addition IL-8, platelet-derived growth factor, endothelial growth factor, and transforming growth factor-␤ were not elevated in the cerebrospinal fluid, suggesting a different signaling pathway and mechanism of angiogenesis. 3 Therefore, our findings were not surprising to us.…”

Familial Association of Basilar Bifurcation Aneurysm and Moyamoya Disease-Four Case Reports-

“…4) Basic fibroblast growth factor and transforming growth factor-b 1 may also be associated with the pathogenesis of moyamoya disease. 5) The present family included patients with familial aneurysm and familial moyamoya disease, a very unusual and so far unreported pedigree. Basilar bifurcation aneurysms were present in two members of this family, moyamoya disease in one, and both basilar bifurcation aneurysm and moyamoya disease were presented in one.…”