Tsuzuki K, Fukatsu R, Takamaru Y, Yoshida T, Hayashi Y, Kobayashi K, Fujii N, Takahata N
Department of Microbiology, Sapporo Medical University, School of Medicine, Japan.
Int J Exp Pathol. 1997 Feb;78(1):1-8. doi: 10.1046/j.1365-2613.1997.9950333.x.
Pathological and immunopathological studies were carried out on snake coiled fibres (SCF) which occurred in affected soleus muscle in chloroquine treated rats. The SCF began to appear in denervated soleus muscle by 8 days after chloroquine injection. By day 14, typical SCF were observed with an unusual swirling pattern of the myofibrils, presenting a bizarre appearance. By day 21 or later, the SCF became less remarkable, and were fragmented and broken apart to form large vacuoles. Immunopathological studies demonstrated that the amyloid beta (A beta) and N and C-terminal regions of amyloid precursor protein (APP), and the amyloid associated proteins tested, apolipoprotein E (apoE), SP-40,40, alpha 1-antichymotrypsin (alpha 1-ACT), and ubiquitin, which are known to be components of amyloid depositions found in Alzheimer's disease (AD) affected brains, were present in the SCF. ApoE, SP-40,40, alpha 1-ACT, and ubiquitin are induced following certain cell challenges (e.g. heat shock, various drugs and injury). The significance of APP, A beta, and amyloid associated proteins are discussed in respect to snake coiled fibre formations in chloroquine rat myopathy and in the amyloidogenesis of AD.
对氯喹处理的大鼠受影响的比目鱼肌中出现的蛇形卷曲纤维（SCF）进行了病理学和免疫病理学研究。在注射氯喹后8天，SCF开始出现在去神经的比目鱼肌中。到第14天，观察到典型的SCF，肌原纤维呈现出异常的漩涡状，外观怪异。到第21天或更晚，SCF变得不那么明显，破碎并分裂形成大的空泡。免疫病理学研究表明，淀粉样β蛋白（Aβ）、淀粉样前体蛋白（APP）的N端和C端区域，以及所检测的淀粉样相关蛋白，载脂蛋白E（apoE）、SP-40,40、α1-抗糜蛋白酶（α1-ACT）和泛素，这些已知是阿尔茨海默病（AD）患者受影响大脑中淀粉样沉积的成分，在SCF中也有存在。ApoE、SP-40,40、α1-ACT和泛素在某些细胞应激（如热休克、各种药物和损伤）后会被诱导产生。本文讨论了APP、Aβ和淀粉样相关蛋白在氯喹诱导的大鼠肌病中蛇形卷曲纤维形成以及AD淀粉样蛋白生成过程中的意义。
