关键词: <, p>, PrP106-126 毒性多肽, 原代神经元, 病理性损伤<, /p>

Abstract: Abstract: To investigate the neuropathological damage of prion diseases，we observed the apoptosis-associated protein changes by immunoblottingand the morphological changes of primary cortical neurons ( PCCN) exposed to PrP106-126 by transmission electron microscopy ( TEM) ． Furthermore，PrP106-126-stimulated primary neuronal death was verified by TUNEL and Annexin V-FITC /PI apoptosis assay． After exposure to PrP106-126 for 24 h，the PCCN showed ultrastructural pathology similar to typical “spongiform vacuoles”in TSEs． A large number of vacuoles of various sizes and swollen and disorganized smooth endoplasmic reticulum ( SEＲ) appeared in the cytoplasm． Nuclear fragments and condensed chromatin were released into the cytoplasm． Consistent with previous researches，we confirmed that PrP106-126 peptide induced mitochondrial cytochrome c release and caspase 3 activation，increased Bcl-2-associated X protein ( BAX) and reduced B-cell lymphoma protein 2 ( Bcl-2) in a timedependent manner． Our observations proved that PrP106-126 is a valid model for researching PrPSc neuropathological damage and neurotoxicity，which provide the theoretical basis for the researchin prion diseases and associated neurodegenerative diseases in vivo.

Key words: <p>PrP106-126, primary cortical neurons, neuropathological changes</p>