Tg(FFI) and Tg(CJD) neurons show different intracellular PrP accumulations and morphological abnormalities of transport organelles.

<p>Cultures of cerebellar granule neurons from non-Tg/<i>Prnp</i>^+/+, Tg(FFI-K5^+/-)/<i>Prnp</i>^0/0 and Tg(CJD-A21^+/-)/<i>Prnp</i>^0/0 mice were fixed and labeled with anti-PrP monoclonal antibody 12B2 using the gold-enhance protocol. WT PrP is mostly found at the plasma membrane (A). D177N/M128 PrP is mostly in the Golgi (B), and D177N/V128 PrP is mostly in the ER, whose cisternae appear enlarged and swollen (C). Scale bar = 250 nm in A, B and C. (D) Quantification of gold particles in different cell compartments. PM, plasma membrane. (E) Quantification of ER and Golgi volumes of cultured cerebellar granule neurons. Data are the mean ± SD of at least 10 cells per specimen. Data for non-Tg/<i>Prnp</i>^+/+ and Tg(CJD-A21^+/-) neurons in D and E are from [<a href="http://www.plospathogens.org/article/info:doi/10.1371/journal.ppat.1004796#ppat.1004796.ref014" target="_blank">14</a>].