We synthesized (trans,trans)-1-bromo-2,5-bis-(3-hydroxycarbonyl-4-hydroxy)styrylbenzene (BSB) and used this compound to detect amyloid fibrils in autopsy and biopsy samples from patients with localized amyloidosis, such as familial prion disease, and systemic amyloidosis, such as familial amyloidotic polyneuropathy, amyloid A (AA) amyloidosis, light chain (AL) amyloidosis, and dialysis-related amyloidosis. BSB showed reactions in all Congo red-positive and immunoreactive regions of the samples examined in the study, and some amyloid fibrils in the tissues could be detected more precisely with BSB than with the other methods. In the mouse model of AA amyloidosis, injected BSB reacted with amyloid in all regions in the serial sections in which Congo red staining was positive. A highly sensitive 27-MHz quartz crystal microbalance analysis revealed that BSB showed a significant affinity for amyloid fibrils purified from familial amyloidotic polyneuropathy and dialysis-related amyloidosis samples and suppressed formation of transthyretin amyloid in vitro. These results suggest that BSB may become a valuable tool for detection of amyloid deposits in amyloidosis and of the mechanism of amyloid formation.

中文翻译：

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一种在体外和体内检测系统性淀粉样变性中淀粉样蛋白沉积的新工具。

我们合成了 (trans,trans)-1-bromo-2,5-bis-(3-hydroxycarbonyl-4-hydroxy)styrylbenzene (BSB) 并使用该化合物检测局部淀粉样变性患者尸检和活检样本中的淀粉样原纤维，例如家族性朊病毒病和系统性淀粉样变性，例如家族性淀粉样变性多发性神经病、淀粉样蛋白 A (AA) 淀粉样变性、轻链 (AL) 淀粉样变性和透析相关性淀粉样变性。BSB 在研究中检查的样品的所有刚果红阳性和免疫反应区域均显示出反应，与其他方法相比，BSB 可以更精确地检测组织中的一些淀粉样蛋白原纤维。在 AA 淀粉样变性小鼠模型中，注射的 BSB 在刚果红染色呈阳性的连续切片的所有区域中与淀粉样蛋白发生反应。高度敏感的 27-MHz 石英晶体微量天平分析表明，BSB 对从家族性淀粉样变性多发性神经病和透析相关淀粉样变性样本中纯化的淀粉样蛋白原纤维具有显着的亲和力，并在体外抑制转甲状腺素蛋白淀粉样蛋白的形成。这些结果表明 BSB 可能成为检测淀粉样变性和淀粉样蛋白形成机制的有价值的工具。