Prion diseases and the BSE crisis (Q41607167)

5 October 2017

1 reference

title

Prion diseases and the BSE crisis (English)

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5 October 2017

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1

Prusiner SB

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5 October 2017

language of work or name

English

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publication date

1 October 1997

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5 October 2017

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5 October 2017

volume

278

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5 October 2017

issue

5336

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5 October 2017

page(s)

245-251

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Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie

5 October 2017

cites work

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Scrapie prions: a three-dimensional model of an infectious fragment

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Is scrapie solely a genetic disease?

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Mice deficient for prion protein exhibit normal neuronal excitability and synaptic transmission in the hippocampus

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Identification of a gene which controls the incubation period of some strains of scrapie agent in mice

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Prion isolate specified allotypic interactions between the cellular and scrapie prion proteins in congenic and transgenic mice

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

NMR structure of the mouse prion protein domain PrP(121-231)

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Recombinant scrapie-like prion protein of 106 amino acids is soluble

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Experimental transmission of BSE and scrapie to the common marmoset

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Characteristics of a Short Incubation Model of Scrapie in the Golden Hamster

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Conversion of truncated and elongated prion proteins into the scrapie isoform in cultured cells

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Immunologic and molecular biologic studies of prion proteins in bovine spongiform encephalopathy

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Pro----leu change at position 102 of prion protein is the most common but not the sole mutation related to Gerstmann-Sträussler syndrome

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Fibrils from brains of cows with new cattle disease contain scrapie-associated protein

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Non-genetic propagation of strain-specific properties of scrapie prion protein.

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Creutzfeldt-Jakob disease in England and Wales, 1980-1984: a case-control study of potential risk factors

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

A comparison of some biological characteristics of the mouse-passaged scrapie agents, 22A and ME7

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Linkage of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease to the prion protein gene

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Transmission dynamics and epidemiology of BSE in British cattle

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

The disease characteristics of different strains of scrapie in Sinc congenic mouse lines: implications for the nature of the agent and host control of pathogenesis

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Inherited prion disease with 144 base pair gene insertion. 1. Genealogical and molecular studies

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Creutzfeldt-Jakob disease: a case-control study

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Scrapie prions aggregate to form amyloid-like birefringent rods

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

The genomic identity of different strains of mouse scrapie is expressed in hamsters and preserved on reisolation in mice

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Natural infection of Suffolk sheep with scrapie virus

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Creutzfeldt-Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Unaltered susceptibility to BSE in transgenic mice expressing human prion protein.

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Spontaneous neurodegeneration in transgenic mice with mutant prion protein

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Kuru--a subacute cerebellar degeneration. The natural history and clinical features

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Klinische und genealogische Beobachtungen bei einem Fall von spastischer Pseudosklerose Jakobs

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

The sequential development of the brain lesion of scrapie in three strains of mice

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques.

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome with an analysis of the various forms of amyloid plaque deposition in the virus-induced spongiform encephalopathies

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Swaledale sheep affected by natural scrapie differ significantly in PrP genotype frequencies from healthy sheep and those selected for reduced incidence of scrapie

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Characterization of detergent-insoluble complexes containing the cellular prion protein and its scrapie isoform

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Transmission of the BSE agent to mice in the absence of detectable abnormal prion protein

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Prion protein mutation in family first reported by Gerstmann, Sträussler, and Scheinker

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

The risk of developing Creutzfeldt-Jakob disease in subjects with the PRNP gene codon 200 point mutation

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Identification of five allelic variants of the sheep PrP gene and their association with natural scrapie

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Frequencies of PrP gene variants in healthy cattle and cattle with BSE in Scotland

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie.

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

The epidemiology of Creutzfeldt-Jakob disease: conclusion of a 15-year investigation in France and review of the world literature

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Fatal familial insomnia and familial Creutzfeldt-Jakob disease: different prion proteins determined by a DNA polymorphism

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker's syndrome

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Diversity of oligosaccharide structures linked to asparagines of the scrapie prion protein

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Pathogenesis of Scrapie Virus Infection in the Mouse

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Experimental infection of cattle with the agents of transmissible mink encephalopathy and scrapie

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Creutzfeldt-Jakob disease and lyophilised dura mater grafts: report of two cases.

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Prion protein is necessary for normal synaptic function

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Self-seeded fibers formed by Sup35, the protein determinant of [PSI+], a heritable prion-like factor of S. cerevisiae.

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Molecular assessment of the potential transmissibilities of BSE and scrapie to humans.

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

BSE transmission to macaques

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Mice devoid of PrP are resistant to scrapie

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Kuru with incubation periods exceeding two decades

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

The new variant form of Creutzfeldt-Jakob disease: a novel prion protein amyloid disorder

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Association between natural scrapie and PrP genotype in a flock of Suffolk sheep in Scotland

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Purification and properties of the cellular and scrapie hamster prion proteins

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Regional mapping of prion proteins in brain

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Analysis of the prion protein gene in thalamic dementia

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

A 'unified theory' of prion propagation

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Sensitive enzyme-linked immunosorbent assay for detection of PrP(Sc) in crude tissue extracts from scrapie-affected mice

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Semipreparative chromatographic method to purify the normal cellular isoform of the prion protein in nondenatured form.

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Different forms of the bovine PrP gene have five or six copies of a short, G-C-rich element within the protein-coding exon

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

The protein product of the het-s heterokaryon incompatibility gene of the fungus Podospora anserina behaves as a prion analog

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

NMR characterization of the full-length recombinant murine prion protein, mPrP(23-231).

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication.

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Measurement of the scrapie agent using an incubation time interval assay

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Transmission of bovine spongiform encephalopathy and scrapie to mice

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Predicted alpha-helical regions of the prion protein when synthesized as peptides form amyloid

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Glycolipid-anchored proteins in neuroblastoma cells form detergent-resistant complexes without caveolin

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Asparagine-linked glycosylation of the scrapie and cellular prion proteins

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes.

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Scrapie strains retain their distinctive characteristics following passages of homogenates from different brain regions and spleen

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Predicting the CJD epidemic in humans

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

A cellular gene encodes scrapie PrP 27-30 protein

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Conformational transitions in peptides containing two putative alpha-helices of the prion protein.

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Unconventional Viruses and the Origin and Disappearance of Kuru

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Phylogenesis of prion protein

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Linkage of prion protein and scrapie incubation time genes

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

"Friendly fire" in medicine: hormones, homografts, and Creutzfeldt-Jakob disease

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Biological evidence that scrapie agent has an independent genome

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Sporadic Creutzfeldt-Jakob disease in a 18-year-old in the UK

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

High-level expression and characterization of a purified 142-residue polypeptide of the prion protein

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Bovine spongiform encephalopathy: epidemiological studies on the origin

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Secondary structure analysis of the scrapie-associated protein PrP 27-30 in water by infrared spectroscopy

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Rapid detection of Creutzfeldt-Jakob disease and scrapie prion proteins

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

[URE3] as an altered URE2 protein: evidence for a prion analog in Saccharomyces cerevisiae

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Scrapie: A transmissible and hereditary disease of sheep

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

In Vitro Propagation of the Prion-Like State of Yeast Sup35 Protein

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Structural clues to prion replication

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

To what extent is strain variation evidence for an independent genome in the agent of the transmissible spongiform encephalopathies?

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Perturbation of the secondary structure of the scrapie prion protein under conditions that alter infectivity

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Molecular biology of prion diseases

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Distinct prion proteins in short and long scrapie incubation period mice

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and the species barrier

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Familial neurological disease associated with spongiform encephalopathy

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Creutzfeldt-Jakob disease from contaminated growth hormone extracts in France

1 reference

11 October 2022

https://opencitations.net/index/coci/api/v1/references/10.1126/SCIENCE.278.5336.245

Identifiers

DOI

10.1126/SCIENCE.278.5336.245

1 reference

5 October 2017

1 reference