TY - JOUR
T1 - Prion protein (PrP) is not involved in the pathogenesis of spongiform encephalopathy in zitter rats
AU - Gomi, Hiroshi
AU - Ikeda, Toshio
AU - Kunieda, Tetsuo
AU - Itohara, Shigeyoshi
AU - Prusiner, Stanley B.
AU - Yamanouchi, Kazuya
N1 - Funding Information:
20 genetically links to the disease and other mutations in Prn are segregated from the variants of familial CJD \[15\]. The findings described here clearly' excluded the possibility that the zitter rat is also the case. We conclude that the zi is not allelic to the Prn gene and that PrP is not involved in the pathogenesis of spongiform encephalopathy in zitter rats. Thus, the zitter rats provides an animal model of spongiform encephalopathy without participation of PrE This work was supported by a Grant-in-Aid from the Ministry of Health and Welfare and from the Ministry of Education, Science and Culture of Japan.
PY - 1994/1/31
Y1 - 1994/1/31
N2 - In order to elucidate the relationship between the prion protein (PrP) structure and the development of spongiform encephalopathy in zitter rats, we analyzed the nucleotide sequences and restriction fragment length variation (RFLV) of the Prn gene encoding PrP in zitter rats and inbred SD/J rats as a control. Prn genes from two strains had identical nucleotide sequences in their coding sequences. Obvious RFLV on the locus was not detected in zitter rats by a Southern blot hybridization. Consistently, zitter rat brains express the normal cellular PrP (PrPC), but do not accumulate the protease-resistant modified isoform (PrPSC). These results indicate that PrP is not involved in the pathogenesis of spongiform encephalopathy in zitter rats.
AB - In order to elucidate the relationship between the prion protein (PrP) structure and the development of spongiform encephalopathy in zitter rats, we analyzed the nucleotide sequences and restriction fragment length variation (RFLV) of the Prn gene encoding PrP in zitter rats and inbred SD/J rats as a control. Prn genes from two strains had identical nucleotide sequences in their coding sequences. Obvious RFLV on the locus was not detected in zitter rats by a Southern blot hybridization. Consistently, zitter rat brains express the normal cellular PrP (PrPC), but do not accumulate the protease-resistant modified isoform (PrPSC). These results indicate that PrP is not involved in the pathogenesis of spongiform encephalopathy in zitter rats.
KW - Polymerase chain reaction
KW - Prion protein
KW - Prn
KW - Spongiform encephalopathy
KW - Zitter rat
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U2 - 10.1016/0304-3940(94)90478-2
DO - 10.1016/0304-3940(94)90478-2
M3 - Article
C2 - 7909925
AN - SCOPUS:0027979911
VL - 166
SP - 171
EP - 174
JO - Neuroscience Letters
JF - Neuroscience Letters
SN - 0304-3940
IS - 2
ER -