Human prion protein with valine 129 prevents expression of variant CJD phenotype

Science

Volumn 306, Issue 5702, 2004, Pages 1793-1796

  Wadsworth, Jonathan D F ^a   Asante, Emmanuel A ^a   Desbruslais, Melanie ^a   Linehan, Jacqueline M ^a   Joiner, Susan ^a   Gowland, Ian ^a   Welch, Julie ^a   Stone, Lisa ^a   Lloyd, Sarah E ^a   Hill, Andrew F ^a,b   Brandner, Sebastian ^a   Collinge, John ^a  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

^b UNIVERSITY OF MELBOURNE   (Australia)

Author keywords

[No Author keywords available]

Indexed keywords

DISEASES; PATHOLOGY;

BOVINE SPONGIFORM ENCEPHALOPATHY (BSE); MOLECULAR PHENOTYPE; TRANSGENIC MICE; VARIANT CREUTZFELDT-JAKOB DISEASE (VCJD);

PROTEINS;

METHIONINE; PRION PROTEIN; VALINE;

PRION DISEASE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CONTROLLED STUDY; CREUTZFELDT JAKOB DISEASE; GENETIC POLYMORPHISM; GENOTYPE; MOUSE; NONHUMAN; PHENOTYPE; PRION DISEASE; PRIORITY JOURNAL; PROTEIN EXPRESSION; TRANSGENIC MOUSE;

AMYLOID; ANIMALS; BRAIN; CATTLE; CREUTZFELDT-JAKOB SYNDROME; ENCEPHALOPATHY, BOVINE SPONGIFORM; HUMANS; METHIONINE; MICE; MICE, TRANSGENIC; PHENOTYPE; POLYMORPHISM, GENETIC; PRIONS; PROTEIN CONFORMATION; PROTEIN PRECURSORS; PRPC PROTEINS; PRPSC PROTEINS; VALINE;

ANIMALIA; BOVINAE; BOVINE SPONGIFORM ENCEPHALOPATHY PRION; MUS MUSCULUS;

EID: 10044224475     PISSN: 00368075     EISSN: None     Source Type: Journal    
DOI: 10.1126/science.1103932     Document Type: Article

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23. We thank C. Brown and his team for animal care, R. Young for preparation of figures, and K. Fox and S. Cooper for technical assistance. We especially thank all patients and their families for generously consenting to use of human tissues in this research, and the UK neuropathologists who have kindly helped in providing these tissues. We thank R. Bradley, D. Matthews, S. A. C. Hawkins and colleagues at the UK Veterinary Laboratories Agency for providing BSE tissues. This work was funded by the UK Medical Research Council and European Commission. One of the routine antibodies used in this work (ICSM 35) is marketed by D-Gen Ltd., an academic spin-off company. J.C. is a director of D-Gen and J.C., J.D.F.W., and A.F.H. are shareholders and consultants of D-Gen.