Microcebus murinus retina: A new model to assess prion-related neurotoxicity in primates

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• 作者：Joan Torrent ; Chadi Soukkarieh ; Guy Lenaers ; Carl Arndt ; Valé ; rie Forster ; Serge Picaud ; Nadine Mestre-Francé ; s ; Jean-Michel Verdier
• 关键词：Prion ; Amyloid fibril ; Neurotoxicity ; Primate
• 刊名：Neurobiology of Disease
• 出版年：2010
• 出版时间：August 2010
• 年：2010
• 卷：39
• 期：2
• 页码：211-220
• 全文大小：2139 K

文摘

No effective treatment currently exists for prion diseases and therefore the development of experimental non-human primate models of prion neurotoxicity, to better understand the underlying mechanism and to test new treatments relevant to humans, represents an urgent medical need. However, the establishment of such models is challenging due to animal welfare and cost considerations. We describe here the use of Microcebus murinus retina, in primary cultures and in vivo, as a new experimental primate model to rapidly examine the effects in the central nervous system of PrP^106–126, a neurotoxic fragment of the human prion protein. We demonstrate that PrP^106–126 triggered rod photoreceptor cell loss by apoptosis and a change in morphology of microglial cells in mixed neuronal–glial cultures of retinal cells. In addition, 2 days after intravitreal injection of PrP^106–126, retinas showed a significant increase in the number of apoptotic nuclei, mainly in the ganglion cell layer.