Gohel C, Grigoriev V, Escaig-Haye F, Lasmézas C I, Deslys J P, Langeveld J, Akaaboune M, Hantaï D, Fournier J G
INSERM U.153, Institut de Myologie, Hôpital de la Salpêtrière, Paris, France.
J Neurosci Res. 1999 Jan 15;55(2):261-7. doi: 10.1002/(SICI)1097-4547(19990115)55:2<261::AID-JNR14>3.0.CO;2-I.
We examined the localization of the normal cellular isoform of prion protein (PrPc) in mammalian skeletal muscle. Using two anti-PrP antibodies, the neuromuscular junction (NMJ) was preferentially stained after immunohistofluorescence. The mouse, hamster, and human NMJ displayed a fluorescent signal specific for PrPc. Postembedding immunoelectron microscopy analysis performed in the mouse muscle showed that the PrPc-specific colloidal gold immunolabelling was concentrated over the sarcoplasmic cytoplasm. The membrane of the postsynaptic domain was devoid of gold particles, while a weak signal was occasionally observed close to the presynaptic vesicles of the terminal axons. These results indicate that the PrP gene is expressed in mammalian muscle at the NMJ. The subsynaptic sarcoplasm of the NMJ appears to be the privileged site where PrPc presumably associated with endosome membrane may play a role in either physiological activity or maintenance of the morphological integrity of the synapse.
我们研究了朊病毒蛋白（PrPc）正常细胞异构体在哺乳动物骨骼肌中的定位。使用两种抗PrP抗体，免疫荧光后神经肌肉接头（NMJ）被优先染色。小鼠、仓鼠和人类的NMJ显示出对PrPc特异的荧光信号。在小鼠肌肉中进行的包埋后免疫电子显微镜分析表明，PrPc特异性胶体金免疫标记集中在肌浆细胞质上。突触后区域的膜没有金颗粒，而偶尔在终末轴突的突触前小泡附近观察到微弱信号。这些结果表明PrP基因在哺乳动物肌肉的NMJ处表达。NMJ的突触下肌浆似乎是一个特殊部位，推测与内体膜相关的PrPc可能在突触的生理活动或形态完整性维持中发挥作用。
