Prion-induced amyloid heart disease with high blood infectivity in transgenic mice

被引：50

作者：

Trifilo, Matthew J.

Yajima, Toshitaka

Gu, Yusu

Dalton, Nancy

Peterson, Kirk L.

Race, Richard E.

Meade-White, Kimberly

Portis, John L.

Masliah, Eliezer

Knowlton, Kirk U. ^{[1
]}

Chesebro, Bruce

Oldstone, Michael B. A.

机构：

[1] Univ Calif San Diego, Dept Med, La Jolla, CA 92093 USA

[2] Scripps Res Inst, Dept Mol & Integrat Neurosci, Viral Immunobiol Lab, La Jolla, CA 92037 USA

[3] Scripps Res Inst, Dept Infectol, Viral Immunobiol Lab, La Jolla, CA 92037 USA

[4] NIAID, Rocky Mt Labs, Persistent Viral Dis Lab, Hamilton, MT 59840 USA

[5] Univ Calif San Diego, Dept Neurosci, La Jolla, CA 92093 USA

[6] Univ Calif San Diego, Dept Pathol, La Jolla, CA 92093 USA

来源：

SCIENCE | 2006年 / 313卷 / 5783期

关键词：

CREUTZFELDT-JAKOB-DISEASE; TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY; DIASTOLIC DYSFUNCTION; SCRAPIE; TRANSFUSION; FAILURE; COMPONENTS; DIAGNOSIS; VARIANT; HUMANS;

D O I：

10.1126/science.1128635

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

We investigated extraneural manifestations in scrapie-infected transgenic mice expressing prion protein lacking the glycophosphatydylinositol membrane anchor. In the brain, blood, and heart, both abnormal protease-resistant prion protein (PrPres) and prion infectivity were readily detected by immunoblot and by inoculation into nontransgenic recipients. The titer of infectious scrapie in blood plasma exceeded 10(7) 50% infectious doses per milliliter. The hearts of these transgenic mice contained PrPres-positive amyloid deposits that led to myocardial stiffness and cardiac disease.

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页码：94 / 97

页数：4

相关论文

共 27 条

[1] Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease [J].

Alperovitch, A ;

Zerr, I ;

Pocchiari, M ;

Mitrova, E ;

Cuesta, JD ;

Hegyi, I ;

Collins, S ;

Kretzschmar, H ;

van Duijn, C ;

Will, RG .

LANCET, 1999, 353 (9165) :1673-1674

[2] Prion-associated dilated cardiomyopathy [J].

Ashwath, ML ;

DeArmond, SJ ;

Culclasure, T .

ARCHIVES OF INTERNAL MEDICINE, 2005, 165 (03) :338-340

[3] The cardiovascular physiologic actions of urocortin II: Acute effects in murine heart failure [J].

Bale, TL ;

Hoshijima, M ;

Gu, Y ;

Dalton, N ;

Anderson, KR ;

Lee, KF ;

Rivier, J ;

Chien, KR ;

Vale, WW ;

Peterson, KL .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2004, 101 (10) :3697-3702

[4] Prions in skeletal muscle [J].

Bosque, PJ ;

Ryou, C ;

Telling, G ;

Peretz, D ;

Legname, G ;

DeArmond, SJ ;

Prusiner, SB .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2002, 99 (06) :3812-3817

[5] Further studies of blood infectivity in an experimental model of transmissible spongiform encephalopathy, with an explanation of why blood components do not transmit Creutzfeldt-Jakob disease in humans [J].

Brown, P ;

Cervenáková, L ;

McShane, LM ;

Barber, P ;

Rubenstein, R ;

Drohan, WN .

TRANSFUSION, 1999, 39 (11-12) :1169-1178

[6] The distribution of infectivity in blood components and plasma derivatives in experimental models of transmissible spongiform encephalopathy [J].

Brown, P ;

Rohwer, RG ;

Dunstan, BC ;

MacAuley, C ;

Gajdusek, DC ;

Drohan, WN .

TRANSFUSION, 1998, 38 (09) :810-816

[7] LEVELS OF INFECTIVITY IN THE BLOOD THROUGHOUT THE INCUBATION PERIOD OF HAMSTERS PERIPHERALLY INJECTED WITH SCRAPIE [J].

CASACCIA, P ;

LADOGANA, A ;

XI, YG ;

POCCHIARI, M .

ARCHIVES OF VIROLOGY, 1989, 108 (1-2) :145-149

[8] Detection of prions in blood [J].

Castilla, J ;

Saá, P ;

Soto, C .

NATURE MEDICINE, 2005, 11 (09) :982-985

[9] Similar levels of infectivity in the blood of mice infected with human-derived vCJD and GSS strains of transmissible spongiform encephalopathy [J].

Cervenakova, L ;

Yakovleva, O ;

McKenzie, C ;

Kolchinsky, S ;

McShane, L ;

Drohan, WN ;

Brown, P .

TRANSFUSION, 2003, 43 (12) :1687-1694

[10] Anchorless prion protein results in infectious amyloid disease without clinical scrapie [J].

Chesebro, B ;

Trifilo, M ;

Race, R ;

Meade-White, K ;

Teng, C ;

LaCasse, R ;

Raymond, L ;

Favara, C ;

Baron, G ;

Priola, S ;

Caughey, B ;

Masliah, E ;

Oldstone, M .

SCIENCE, 2005, 308 (5727) :1435-1439