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| ACCUMULATION OF PROTEINASE K-RESISTANT PRION PROTEIN (PRP) IS RESTRICTED BY THE EXPRESSION LEVEL OF NORMAL PRP IN MICE INOCULATED WITH A MOUSE-ADAPTED STRAIN OF THE CREUTZFELDT-JAKOB-DISEASE AGENT |
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| Author(s): SAKAGUCHI S, KATAMINE S, SHIGEMATSU K, NAKATANI A, MORIUCHI R, NISHIDA N, KUROKAWA K, NAKAOKE R, SATO H, JISHAGE K, KUNO J, NODA T, MIYAMOTO T |
| Source: JOURNAL OF VIROLOGY Volume: 69 Issue: 12 Pages: 7586-7592 Published: DEC 1995 |
| Times Cited: 85 References: 37 |
| Abstract: Creutzfeldt-Jakob disease (CJD) is a transmissible neurodegenerative disease of humans caused by an unidentified infectious agent, the prion. To determine whether there was an involvement of the host-encoded prion protein (PrPc) in CJD development and prion propagation, mice heterozygous (PrP+/-) or homozygous (PrP-/-) for a disrupted PrP gene were established and inoculated with the mouse-adapted CJD agent. In keeping with findings of previous studies using other lines of PrP-less mice inoculated with scrapie agents, no PrP-/- mice showed any sign of the disease for 460 days after inoculation, while all of the PrP+/- and control PrP+/+ mice developed CJD-like symptoms and died. The incubation period for PrP+/- mice, 259 +/- 27 days, was much longer than that for PrP+/+ mice, 138 +/- 12 days. Propagation of the prion was barely detectable in the brains of PrP-/- mice and was estimated to be at a level at least 4 orders of magnitude lower than that in PrP+/+ mice. These findings indicate that PrPc is necessary for both the development of the disease and propagation of the prion in the inoculated mice. The proteinase-resistant PrP (PrPres) was undetectable in the brain tissues of the inoculated PrP-/- mice, while it accumulated in the affected brains of PrP+/+ and PrP+/- mice. Interestingly, the maximum level of PrPres in the brains of PrP+/- mice was about half of the level in the similarly affected brains of PrP+/+ mite, indicating that PrPres accumulation is restricted by the level of PrPc. |
| Document Type: Article |
| Language: English |
Addresses:
1. NAGASAKI UNIV, SCH MED, DEPT BACTERIOL, NAGASAKI 852, JAPAN
2. NAGASAKI UNIV, SCH MED, DEPT PATHOL 2, NAGASAKI 852, JAPAN
3. NAGASAKI UNIV, SCH MED, LAB ANIM CTR BIOMED RES, NAGASAKI 852, JAPAN
4. INST CANC RES, DEPT CELL BIOL, TOSHIMA KU, TOKYO 170, JAPAN |
| Publisher: AMER SOC MICROBIOLOGY, 1325 MASSACHUSETTS AVENUE, NW, WASHINGTON, DC 20005-4171 |
| Subject Category: Virology |
| IDS Number: TE366 |
| ISSN: 0022-538X |
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| | | | | | | | | Record from Web of Science® | | | | | | | | | |