Profoundly different prion diseases in knock-in mice carrying single PrP codon substitutions associated with human diseases

Proceedings of the National Academy of Sciences of the United States of America

Volumn 110, Issue 36, 2013, Pages 14759-14764

  Jackson, Walker S ^a,b,e   Borkowski, Andrew W ^a,b   Watson, Nicki E ^a   King, Oliver D ^c   Faas, Henryk ^d   Jasanoff, Alan ^b,d   Lindquist, Susan ^a,b  

^a WHITEHEAD INSTITUTE FOR BIOMEDICAL RESEARCH   (United States)

^b MASSACHUSETTS INSTITUTE OF TECHNOLOGY   (United States)

^c UNIVERSITY OF MASSACHUSETTS MEDICAL SCHOOL   (United States)

^d MASSACHUSETTS INSTITUTE OF TECHNOLOGY   (United States)

^e GERMAN CENTER FOR NEURODEGENERATIVE DISEASES DZNE   (Germany)

Author keywords

Neurodegeneration; Protein aggregation; Protein misfolding; Transgenic mice

Indexed keywords

AMINO ACID; PRION PROTEIN; PROTEINASE K; CODON; CYCLINE; PRION;

ALLELE; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN SPONGIOSIS; CEREBELLUM; CODON; CONTROLLED STUDY; CREUTZFELDT JAKOB DISEASE; FATAL FAMILIAL INSOMNIA; GENE; GENOME; GLIOSIS; HIPPOCAMPUS; HUMAN; MOUSE; MUTATION; NONHUMAN; PRION DISEASE; PRIORITY JOURNAL; THALAMUS; 129 MOUSE; ANIMAL; BIOLOGICAL MODEL; BRAIN; C57BL MOUSE; DISEASE MODEL; FEMALE; GENETICS; IMMUNOHISTOCHEMISTRY; KAPLAN MEIER METHOD; KNOCKOUT MOUSE; MALE; METABOLISM; PATHOLOGY; PHENOTYPE; PRION; TRANSGENIC MOUSE; TRANSMISSION; WESTERN BLOTTING;

MUS; MUS MUSCULUS;

ANIMALS; BLOTTING, WESTERN; BRAIN; CODON; CREUTZFELDT-JAKOB SYNDROME; DISEASE MODELS, ANIMAL; FEMALE; HUMANS; IMMUNOHISTOCHEMISTRY; INSOMNIA, FATAL FAMILIAL; KAPLAN-MEIER ESTIMATE; MALE; MICE; MICE, 129 STRAIN; MICE, INBRED C57BL; MICE, KNOCKOUT; MICE, TRANSGENIC; MODELS, GENETIC; MUTATION; PHENOTYPE; PRION DISEASES; PRIONS; PROLIFERATING CELL NUCLEAR ANTIGEN;

EID: 84883350837     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.1312006110     Document Type: Article

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