Prion disease
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Human genome project and disease genes:
.. matching genes to inherited diseases
.. human genomics
Conformational diseases (amyloidoses):
.. cross-beta disorders
.. congophilic fibrils
Prion and doppel genes:
.. chromosome 20 context
.. doppel directory
.. prion exons and introns
.. prion mRNA regulation
.. familial CJD
.. prion genes in other species
.. evolution 
Prion protein:
.. covalent modification, repeat region, copper, 106-126
.. 3D structure, conformation, protein-protein interactions
.. species barrier, strain types, cell biology
.. normal function 
Specific TSEs:
.. sporadic CJD, nvCJD, iatrogenic CJD, diagnosis, therapy 
.. BSE, scrapie, TME, Zoo BSE, CWD
General interest:
.. meetings, books, people, historical aspects

Human genomics

1,525,050 bp human chr 20p contig released
Microsatellites in prion-doppel region useful in kindred tracking.
NT_001001: largest finished contig at GenBank containing prion-doppel
SNPs in prion-doppel region: single nucleotide polymorphic markers
Human genome browser: prion flanking genes (chr20:4844409-5855419)
Another case of intergenic splicing on human chr 1
Distant prion relative in Drosophila?
Prion missing from complete Drosophila genome
Jag2: another prion gene on chromosome 14?
Normal function: a lesson from frataxin
Human genome -- still an unfinished mess; ADRA1A is 5' prion neighbor

Matching genes to inherited human diseases

Partly mapped human disease genes
Using table and Genome Browser to match inherited disease with gene
How the table was made
How the table will be used
How the table will be validated
How the table will be kept current
Quality control: checks that were run

Cross-beta conformational disorders (amyloidoses)

Insulin: the first infectious amyloidosis
Infectivity of Alzheimer seeding
N-methylation - a general strategy to prevent amyloid formation
Inhibition of huntingtin fibrillogenesis by Congo red etc.
Amyloid plaques found in aging salmon
Familial Danish dementia
Coexistence of Alzheimer and prion amyloid
Blockers for orphan amyloid diseases
Yeast amyloid fibril: second gene influences fibril initiation
Amyloid channel toxicity: prion, AD, islet amylin, poly-glutamine
Universal architecture for amyloid diseases
Acylphosphatase: can any protein form amyloid protofilaments and fibrils?
Earliest recognition of cross-beta architecture
JW Kelly on conformational disease
Quaternary structure and amyloid
Inouye on prion amyloid structure
A systematic search for repeat conformational disorders
Genome-wide screens for polyglutamine disease
Images of prion fibrils and fibers
Molecular model of Alzheimer fibril
Details on specific amyloid proteins
Similarities between inclusion-body myositis, Alzheimer, and prion disease
Triplet repeat diseases: innocent inclusions?
Deliberate domino iatrogenic amyloidosis
Neurological repeat diseases
Repeat diseases: repeat not involved in oligomerization Amyotrophy revisited
Fragment-enhancing Alzheimer mutation
Huntingon: seeded fibril nucleation
Familial British dementia: read-thru of stop codon in cerebral amyloid angiopathy
Familial dementia: loop insertion polymerization of neuroserpin
Hereditary cardiac amyloidosis
Transmission/induction of another amyloidosis by diet
Structure of SH3 cross beta fibril
Met/met at 129 predisposes to sporadic inclusion-body myositis
Familial Mediterranean Fever: indirect serum amyloid
Friedreich ataxia: non-amyloid intronic polyGAA repeat
Spinobulbar muscular atrophy -- X-linked androgen amyloid?
New human amyloid identified
Oculopharyngeal dystrophy: autosomal dominant poly-alanine expansion
Wernicke encephalopathy (alcoholism) and CJD: chicken or egg?
Poly-gln domain of drosophila GAGA forms amyloid fibre
Amyloid found in bacteriophage tailspike P22
Huntingtin amyloid or transglutaminase cross link?
Familiar familial prionlike disorders
Autosomal dominant 3' UTR CTG repeat expansion in SCA 8 and DM
New familial encephalopathy with neuroserpin inclusion bodies
A30P and A53T amyloid filaments of alpha-synuclein (NAC) in PD
Phenotypic heterogeneity of FTDP-17: alternative splicing increases 4 repeat tau
Exposure of cryptic epitopes on transthyretin only in amyloidogenic mutants
Fibrinogen alpha amyloid: renal transplant 'infected'
Mutant ubiquitinylation tied to brain diseases
Most Parkinson's cases sporadic
Gamma-secretase enahncement of amyloid precursor protein
Exposure of cryptic epitopes on transthyretin only in amyloid
New neurodegeneration gene (HSS) maps by prion gene
Transthyretin amyloid
Inhibiting transthyretin conformational changes
An unusual case of CJD: "Parkinson plus"
Neuronal storage disease and CJD
Huntington's Disease breakthrough: quotes from the journals
Friedreich's ataxia homologue found in yeast
PPND and FTDP as tau repeat diseases
Mutations in Fanconi anemia gene similar to prion
SH3 joins the amyloid club
Amyloid fibril formation by an SH3 domain
Cross-beta filaments formed by leucine-rich repeat peptides
Cerebellar leucine-rich ANP interacts with ataxin-1 of SCA-1
Leucine rich repeats: cross-beta yes, but no disease yet
Cause of brain cell death found for 6 diseases
Amyloidosis causing a progressive myopathy
Model peptide transitions in vitro
Serum amyloid A: critical amyloid regions
Cerebral amyloid angiopathy review
Interstitial amyloid deposits in the pituitary gland
Membrane phospholipid seeds amyloid formation
Multiple isoforms of bovine serum amyloid-A (apoSAA)
New prion analog found
Transcriptional errors accumulate about GAGAG
Apo E4 and Clusterin
Apolipoprotein E and A-I genes
AD amyloid peroxide causes Alzheimer's damage?
APO E not correlated with CJD
Alzheimer's and CJD
Alzheimer-related genetic factor linked to brain damage in boxers
Alzheimer's: New Lesion Found in Diseased Brains
New plaque protein identified in brains of people with Alzheimer's disease
How much Alzheimer is really CJD?
How good are neurologists at CJD diagnosis anyway?
Alzheimer Resources
Chlamydia pneumoniae in Alzheimer glial cells
Alzheimer researchers net zebrafish gene
Gene affecting early onset of Alzheimer's
CJD-Alzheimer similarity?
Conformational mimicry in Alzheimer's disease
Non-A beta component of Alzheimer's disease amyloid (NAC) is amyloidogenic
Extra repeat mutations in tau
Blood brain barrier in Alzheimer
Angiopathies masquerading as Alzheimer's disease?
C. elegans presenilin functionally redundant with presenilin
Alzheimer's disease: The ins and outs of amyloid-beta
New gene linked to Alzheimer's is found
Two reviews of genetic neurodegenerative diseases
Alzheimer-CJD cross-over
Two more genes for Alzheimer's disease
Alzheimer and PD
Cytotoxicity of prion 106-126 unlike Alzheimer's A beta 25-35
Scientists pinpoint role of Alzheimer's genes Yeast 'prion' developments
Mutational Analysis of Het-s Prion Analog of Podospora anserina
Interplay of yeast psi prion chaperones
Role of Hsp70-related chaperone in yeast psi prion
Equilibrium folding properties of the yeast prion Ure2
Real biological role for yeast prions?
Two prion-inducing regions of ure2p are nonoverlapping
Antagonistic interactions between yeast chaperones Hsp104 and Hsp70 in prion curing.
Mini-review: The Yeast Psi+ Prion: Making Sense of Nonsense
Is there a human psi?
Yeast prion polyglutamine
Mini-review: The Yeast Psi+ Prion: Making Sense of Nonsense
Overproduction of Sup45 inhibits Sup35 seed formation
De novo appearance of PSI+ prion in S. cerevisiae
Differential resistance to proteinase K of yeast Ure2p in vitro
Details on new fungal prion
Latest on yaest Psi fibrils

Congo red birefringence: specific to amyloidoses

The 15 congophilic disorders
Congo red variants distinguish amyloid fibrils
Congo red imagery
Image of Congo red binding to huntingtin aggregate
GG Glenner was right: yeast prions URE3 and Sup35 are congo red birefringent
Congophilic sporadic inclusion-body myositis and myopathy
Recruitment, species barriers, strain types, and infectivity
A universal structure for congophilic amyloid?
Structural aspects of Congo red
Yeast prions form congophilic seed fibers
Parkinson's Disease: are Lewy bodies congophilic?
Congo red prolongs the incubation period in scrapie-infected hamsters Dentatorubralpallidoluysian atrophy: are fibrils congophilic?
NAC is congophilic, may seed Abeta deposition
BIGH3 -- transmissible congophilic corneal amyloid?
Yeast prions form congophilic seed fibers

Chromosome 20

New doppel neighbor: a tough little gene
Microsatellites in the prion-doppel region
5' prion neighbor: ADRA1A (alpha-adrenergic receptor)
Chr 20 genes with unigene links
Details on 4th gene: RPS4X pseudogene
Massive parasitism of chromosome 20
Have any genes been missed?
142,634 genes on human chromosome?
Ancestral origins of E200K -- better chr 20 microsatellites now available
Did a tandem gene doubling create the prion superfamily?
Pseudogene prion neighbor: isopentenyl diphosphate:dimethylallyl diphosphate isomerase IPP
Introduction to genome annotation
30 kb example: a dynein pseudogene
Characterizing features
Pseudogene annotation: the issues Cheap tricks: assembling unfinished contigs
How GeneBander tracks are made
How they did it in the old days
Status and limitations of current tools
Glossary, notation, and acronyms
Published examples of good annotations
Dynein pseudogene consisting solely of 3' UTR
Disease-finding with GenMap00
Gene within a gene
GenMap00: how the map was made
GenMap00: the map
Hallervorden-Spatz syndrome
L7a: A processed pseudogene with retained intron
Nearby diseases: AR CHED, AD CHED, and PPMD
KIAA0168: nearest neighboring gene 3' of prion protein
Prion gene sequence, neigbors, synteny, and pseudogenes
Historic duplications of the prion gene
Human-mouse prion gene synteny relationships
Prion gene syntenies in other species
Synteny references
Chromosomal abnormalities involving the prion gene
Experimental recommendations: role of chromosomal abnormalities
References: Trisomy in chromosome 20p
References: Ring trisomy in chromosome 20p
References: Monosomy in chromosome 20p
References: Chromosome 20 genetic imprinting
Chromosomal organization of 3 prion genes
GenBank: false alarm on chromosome 7
Retrotransposon structure of the prion gene region
Cattle Prion DNA
Prion gene neighbors
100,000 bp of human, mouse, sheep, prion genes
A 3' UTR mariner element is found in cow too
New chromosomal location of prion gene
Mouse IAP retrovirus
Intron sequence alleles and CJD diagnosis
CpG codon depletion
Predictions of new human point mutations from CpG effect

Doppel: tandem paralogue of prion gene

Doppel developments
...human: end of doppel gene found  ... pig:   doppel protein inferred from EST
...rat:   new pre-CDS exon defined  ... cow:   5'coding doppel EST found by USDA
...mouse: cryptic pre-CDS exon      ... mouse: 3'UTR end from EST data
...alignment: doppel 5'UTR splice exon junction from five species
...blastn:    advanced parameter settings for alignment of non-coding exons
...ClustalW:  doppel protein alignment from 6 species
Doppel changing 2.1x faster than prions
Doppel alleles and CpG: ancient tandem prion paralogue.
Doppel polymorphisms in nvCJD: no apparent connection
Doppel bibliography
CpG hotspots for doppel
Index to doppel articles
Doppel protein properties
5 alleles of human doppel
Doppel allele and sequence resourse
CpG hotspots for doppel
Odd new doppel EST
Cow and sheep doppel genes
Index to doppel articles
Doppel disease
Doppel delay of 54 months
Origin of doppel chimeric transcripts
First human doppel allele found: M174T
Doppelcross: cis-trans genetic issues
Doppel/prion alignments: getting them straight
Doppel disulfide in 3D (and related issues)
Glycosylation sites in bird prion and mammalian doppel
Candidate exon 2 switch alleles
Intergenic exon 1 is within a Mer115 insertion element Doppel prion upregulated in knockouts
Prnd and Prnp gobbled up by human genome project
Which came first, the prion or the ghost?
Ghost prion promoters
Bridging the gap
Prion paralogue found adjacent to main gene!
Long and short incubation mouse prion genes compared
Re-doubling of doppel-prion pair
Six new human doppel transcripts
Genes on 187,029 bp of flanking sequence of prion-doppel
"Alternative splicing" across prion-doppel tandem duplication
Effects of a disrupted disulfide loop
Impaired motor coordination in mice lacking prion protein.
Increased sensitivity to seizures in mice prion knockouts

Prion exons and introns

Prion genomics round-up: a human exon 2 EST
Exon 1: 13 sequences from 6 species with flanking regions and splice-typing
Exon1b of cow compared to putative sheep exon 1b
Exon 2 for 6 species: alignment, consensus, distance, fasta, flanking regions
Exon 3: 13 species, alignment, fasta, flanking regions, probes
Evolution of exon 2: ancient region changes slowly
Alu and L1 retrotransposon insertion dating
The G-21A polymorphism
Major Cpg island around exon 1
Supplementary material
A long alignable region within intron 1
Motif mystery upstream of exon 1 promoter
Reference sequences for motif and promoter region
Exon 1-2-3 spliced probe sequences
Annotated References
Exon 2: the mystery deepens
Adjacent genes
Is exon 2 splice-competent?
Exon archive
Bovine prion mRNA exon 1: tissue-specific alternative splicing
Mystery prion peptides and alternative regulation
Repressor found in prion promoter
Promoter polymorphism in Alzheimer

Prion gene expression

3'UTR regulation of mRNA
Prion mRNA in sheep placenta
Prion polyA variation -- does it lead to over-production?
Circadian regulation of prion mRNA: widespread synchronous rhythm.
Anti-prion 4.5 kb mRNA: a harmless prion pseudogene?
Alternate exon 1 splicing in Holstein
Doxycycline control of prion protein
Doxycycline control of prion protein transgene expression
Phosmet induces up-regulation of surface levels of the cellular prion protein
New prion mRNA complexity found
Rapid disease course in 8x prion gene over-expression
Anti-prions resurrected
Making (anti)sense of non-coding sequence conservation
Upregulation of lysosomal hydrolases, perforin, and peroxidases in TSE mice

Familial CJD

Compound and homozygous prion mutations
Predicting missing CJD mutations from CpG effect
Nomenclature and ascertainment issues: recommendations
6 new human prion mutations
Late onset mutations: E196K, V203I, E211Q
Accumulation of protease-resistant prion protein in 9x insertional mutants
Distinct Dutch 7x repeat CJD
CJD profile in patients homozygous for PRNP E200K.
High prevalence of mutations in patients with early-onset dementia
Late onset prion mutations: E196K, V203I, E211Q
T188A prion mutation in an octogenarian with CJD
Germany: 12 generations of "fatal familial insomnia"
H187R: novel PRNP sequence variant associated with familial encephalopathy.
New mutation, H187R, tracks with GSS
Prominent psychiatric features, early onset in a new insertional mutation
9 new prion gene mutations include T188R and P238S
Large new kindred of A117V
French review of FFI: haplotypes and phenotypes
Y145 stop: proteasomal degradation and PK resistance
E200K + valine at codon 129 = type 2 protease-resistant prion
French E200K cluster
Ancestral origin of E200K: 6 founding mutations in 62 families
D178N: first Austrian kindred
New E200K cluster in Japan
P101L in mouse alters TSE transmission time
Known point variations in human prion coding region
Prion genetics review
New CJD mutation disrupts glycosylation: T183A
Cumulative distribution anomaly in known human CJD mutations
K200K homozygotes
Protective polymorphisms: codon 219 is better
Familial CJD at codon 200
New E200K cluster in Japan
N171S reported earlier in normal patient
N171S -- commentary
Prions linked to mental illness
Val/val associated with loss of mental ability
No prion plaque in M232R CJD
Double mutants: is disease onset shorter or the same?
Phenotypic variability of GSS
Phenotypic variability of GSS: new mutations D202N, GSS Q212P
Prion termini in P102L GSS
Phenotypic GSS and new mutations D202N and Q212P
GSS with V129 is very different from GSS with M129
Misfolding in GSS

Prion genes in other species

Prion incubation time QTL in mouse
Quail, pigeon prion mix-up
Bovine and ovine alleles and mutations at CJD-causative sites from CpG effect.
2x prion repeats in lemurs
Turtle prion gene
...Gene organization: 5' UTR, splice acceptor, single exon, 3' UTR
...Signal and GPI peptides
...Turtle repeat: hexapeptide similar to birds
...Invariant region, beta strands, helices, disulfide, glycosylation retained
...Second loop mystery resolved
...EF-hand motif: not really
Evolutionary appearance of turtles relative to birds, mammals
The ancestral amniote prion: alignment of turtle, bird, mammal, and doppel
New sheep alleles
Alignment of sheep and human prion protein
Odd prion allele found in goat
Predicting cow polymorphisms: bad apple bovine theory
Codon use in prion gene versus bovine codon use overall
Amino acid polymorphisms of prions in Japanese and Dutch sheep
Prion gene variation in primates: correction
Harmless polymorphism?
Reference sequences -- color coded
Mule Deer sequence update
Mule deer on hold: AF009180, U97331, AF009181 undergoing revision
Genomic structure of complete bovine PrP gene
Red deer and camel prion sequences updated
White-tailed deer alleles
Pronghorn, fallow deer prion sequences released
Cat sequences in conflict: alignment
Liverpool group posts contradictory cat sequence
Rohwer group posts cat prion
Unusual aspects of cat prion -- Alex Bossers
Cat prion protein predicted
Dog prion anomaly confirmed
Crocodile prion sequence predicted
Introduction to kudu and oryx issues
Comparison of kudu nucleic acid sequences
Comparison of kudu amino acid sequences
DNA and protein sequences of kudu, oryx, sheep, and cow
Kudu references
Oryx and kudu prion sequences

Prion evolution

Displaying residue conservation in the prion protein
Why not just use GenBank?
Quick quality checks of new prion sequences
Putting the database to use: examples
High priority sequencing needs
Common mistakes in prion genomics
Format and structure of the sequence data
Getting exactly the data you need
Prion sequence database update log
Prion gene mapped in horse
Implications of (Tylopoda, (Suidae, (Cetaceae, (chevrotain,..))))
7 new bird prion sequences
27 new mammalian and 9 bird prions: high conservation of flexible region
Guinea pig prion sequence: PCR errors claimed
Zebrafish RH panel locates prion gene
Odd prion allele found in goat
Application of distance geometry to 3D visualization of prion sequence relationships
Analysis of the prion gene: introduction
Feature tables of mammalian prion genes
Making (anti)sense of non-coding sequence conservation
Rat prion cytochrome c pseudogene
Outgroup arbitration applied to prion promoter
Alignment of cyt c pseudogene delimited pre-exon 1
Ancestral size of intron 1
Full length alignment and analysis of cow and sheep
Expanding on the Gibbs Principle
Application: the cetaceaen-artiodactyl divergence
Revised dates of divergence
Phylogeny for the prion protein
Nearly-neutral mutations from primates
Prion Evolution
Prion evolutionary rate
Evolutionary change of proline in mammalian and avian prions
Chicken prion
Nematode genome 85% complete: no prion yet
New jungle species: dwarf marmoset, soala ox, dwarf and giant muntjacs
Molecular evolution in relation to spongiform disease
Online sequence analysis

Covalent modifications

Ragged N terminus in types of CJD
Artefacts: 4-hydroxyproline at Pro 44, polypro II pre-repeat helix
Arginine fiasco
Selective oxidation of prion methionine residues
Control of disulphides in the endoplasmic reticulum and in vitro
Ubiquitinated inclusions in prion diseases
Prion glycan: bittersweet situation
200-truncated PrP is present in mature sperm
Lehmann/Harris: blockade of glycosylation promotes scrapie
Prion glycosylation
Mouse prion glycans determined
Prion oligomers, GPI viral receptors
Microdomains of GPI-anchored proteins
New human GPI protein found
Other GPI Proteins
Two new GPI neural proteins found
No altered aspartyl residues in scrapie
Prion rods contain small amounts of two host sphingolipids
Ubiquitinated inclusions in prion diseases

Repeat Region

Unusual repeat in lemur prion
Cow prion with 7 repeats
Chicken prion tandem repeats form a stable, protease-resistant domain.
Octapeptide insertions/deletions explained by DNA slippage
Genetic mechanism causing CJD insertion mutants
Prion deletion mutants
Are prion deletions a CJD risk?
Flanking region amino acid sequences
Are the longer deletions diseases of normal prion function? Not proven
The avian repeat anomaly
So what is the structure of the repeat region?
Take me to the pdb coordinates
Structure of flanking regions
Review of structural papers
Octapeptide Variability
Octapeptide repeats
Octapeptides in primates
Repeat region amino acid alignment: 80 species
Repeat sequence DNA from 76 species
Sequence variations in the repeat region
Number of extra repeats determines the type of cerebellar deposits
Chicken prion tandem repeats: a stable, protease-resistant domain
Higher repeat properties in cultured cells
Transport, sensory, conditioning roles for repeat region
Other proteins with tandem repeats
Prion repeats compared to yeast S35 repeats
Squalene cyclase and QW motif: relevence to prion repeat structure?
Blast pseudo-homologues of repeat region
3D structure of the repeat domain: introduction
Slippage mechanism of deletions
Deletion References
Supplemental repeat deletion material
Wildtype human reference sequence
Deletions in other species
Phylgeny of deletion slippage
detection of repeats with streak tool
Prion repeat region deletions: introduction
Distinguishing deletions
Frequency of deletion classes: worldwide summary
Predicting the final list of allowed and forbidden OPRI mutations
Repeat References
Insertion alleles in other species
Origin of extra prion repeat units
Disentangling slippage from editing
How many different insertion mutations will eventually be found?
Prion mRNA hairpins
Phylogeny of hairpin C
Hairpin C revisited in more species


Copper-induced asparagine 107 deamidation
Repeat copper: modularity and universality
Copper binding to octarepeat peptides by electrospray ionization mass spec
Manganese replacement of copper in prion protein function and protease K resistance
Brain copper and cuproenzyme activity: variance with prion protein level not confirmed
Repeat region of prion protein reduces copper: role of tryptophan residues.
Copper potentiation of Alzheimer Abeta neurotoxicity
Raman pH dependence of copper binding to prion octapeptide
Prion octapeptide: intermolecular copper
Copper chelators and strain types 1 and 2
Copper stimulates endocytosis of the prions
Copper chaperones: no known homology to prion protein
Copper and the prion protein
Copper: a role for prion protein?
Copper enzymes
Review of known copper-binding proteins
Copper in normal prion
Cu in octapeptide repeats
..suitability of apo-fragment?
..missing controls
..stoichiometry inconsistencies what about GPI-ceruloplasmin?
..where are those cuprione citations?
Copper binding not demonstrated: webmaster
Copper in Alzheimer's disease: histidines in APP 135-155
Comprehensive Dec. 97 review article of copper proteins
Repeat motifs in copper-binding membrane transporters: Menkes and Wilson disease
Earlier copper chelator studies
cuprizone and scrapie
Prion protein 'binds copper in vivo'
Copper stimulates endocytosis of the prions
Copper chaperones: no known homology to prion protein
Copper chaperones in yeast
Prion repeat binds Cu++, tryptophan, glycine carbonyl implicated
Copper binding to extra-cellular domain of Alzheimer APP
Prion repeat copper by mass spec
Copper, GuHCl, and infectivity
Copper ion orders octapeptide repeat region
Copper in SOD

106-126 Amyloidogenic Region

9x repeat not neurotoxic in cultured cells
Inhibition of in vitro conversion by 109 to 141
Variants of cross-beta region 106-126
106-126 isoaspartate?
Flexible region 104-113 studied by epitope-antibody xray
Fibrillization of human prion 90-231
Optimal toxicity: 106-126
A synthetic peptide initiates GSS in transgenic mice
Mini-prion up and running
Clusterin prevents aggregation of neuropeptide 106-126 in vitro.

3D structure

3D modelling of prion glycans and GPI anchor: helix A left for dimer
Warwicker pdb model of domain-swapped dimer
Linear and conformational prion epitopes
Human prion nmr structure
Nmr structure of PrP106-126
Refinement of hamster nmr
Improved NMR structure of Syrian hamster prion
Full length prion nmr structure
3D structure of longer hamster prion
New prion structural studies
Comments on hamster structure
3D mouse prion coordinates finally available?
Hamster pdb coordinates
Hamster coordinate hang-up
Show me the pictures
Amber vs Eng & Huber force fields: are structural issues real?
Table of bad bonds in refined Swiss mouse
hamster coordinates
Best available mouse
Coordinate data: quality control, quality assurance
A few residual issues for Official Swiss Mouse
What does bond strain in prion core mean?
Structure of full-length hamster prion 29-231
Model 10 hamster prion 90-231
Refolding of 121-231
AA Residues 109-121
AA Residues 108-218
Residues 121-231
Swiss NMR group on mammalian prion structure
Coordinates for viewing sheep alleles threaded on mouse
What got modelled in 3D Crunch?
What did 3D Crunch do with opossum and chicken?
Crunch prion sequence alignment and links
3D Crunch to benefit prion research
3D threaded model gallery
Prion protein NMR structure and familial CJD
Prion chameleon helices
It's not a bug, it's a feature
X-ray Diffraction of Scrapie Prion
Interactive prion models


In vitro conditions for prion fibril formation.
Transmissible and genetic prion diseases share a common pathway
Cycling in early endosomes
Protease-resistant, detergent-insoluble prion not necessarily infectious
Rapid acquisition of beta-sheet in prion protein prior to multimer formation.
Protease-resistant prion produced in vitro lacks detectable infectivity.
Conversion of normal prion via a localized domain
Prion picture gallery
Hydrogen bond donors and acceptors
Prion helical wheels
Prion dipoles and conformation
Prion Hydrophobicity
Cis and trans proline in the 3D prion structure
From helix to sheet after proteolysis
In situ formation of rogue prion
In vitro formation of rogue prion
N-terminal conformational change inferred
Conformational intermediates of purified human prion
Protease-resistant prion produced in vitro lacks detectable infectivity
Beta intermediate found for 121-231
Formation of prion fibrils in brain slices
Design of peptides undergoing alpha-to-beta transition and amyloidogenesis
Rapid acquisition of beta-sheet structure in prion prior to multimer formation
Alleles found in rogue conformer
Inhibiting prion conversion with central peptides, 106-141
Deadly Conformations: Protein Misfolding in Prion Disease
Models for the Conversion of PrPC to PrPSc
Misfolding references
Implications for prion-only model
Sheep-to-human and cow-to-human transmission demonstrated in vitro
Full length prion monomer purified in quantity from cow
DNA/protein sequence actually used to make bovine prion
The transgenic mouse experiment: what does it mean?
A model of prion capping in ARR sheep
SAA fibril formations accelerated by injected seed
Hydrophobic patches at low pH
What is an apatamer anyway?
RNA aptamers specifically interact with the prion protein
Show me a graphic of a stackable G-quartet
How aptamers might be used in diagnosis and therapy
Could aptamers be responsible for prion insertion mutations?
Quick commentary on new anti-prion antibody
Prion-specific epitope defined by a monoclonal antibody!
Summary table: who immuno-precipitates what?
Prion immunoreactivity in appendix
Parvalbumin-immunoreactive neurons associated with CJD
Immunodetection of rogue prion in spleens
Monoclonal antibody: conserved epitope on ruminant prion
Prions studied with panel of new monoclonals
New prion antibodies
Accuracy of Figure 3b, 3c questioned

Protein-protein interactions, chaperones

Monomer-dimer equilibrium of native bovine Prpc not seen in recombinant protein
Chaperone BiP binds to mutant prion and mediates proteosome degradation
Prion protein colocalizes with dystroglycan complex in brain
Self-assembly of recombinant prion protein of 106 residues
Chaperone BiP binds to mutant prion and mediates proteosome degradation
Heterologous prion binding, inhibition of conversion, and species barriers
Monomer-dimer equilibrium of native bovine Prpc not seen in recombinant protein
Prion rods contain an inert polysaccharide scaffold.
Protocadherin II: cell surface receptor for normal prion
Altered phospholipase release of prion mutants
Protocadherin backgrounder
Structure of the protocadherin extra-cellular domain
Laminin binds another amyloid former, serum amyloid A.
Imagery of possible rogue prion dimer
Domain-swapping and fibril formation
Prion protein interactions with a synthetic fragment
Identification of candidate proteins binding to prion protein
Surprising prion binding to APP 1 and Nrf 2
Human analogue of scrapie-responsive Gene ScRG-1
Prion dimer interface
Prion membrane interaction: Warwicker
Protein X: interaction with prion protein
Doubts raised on laminin receptor role
Laminin binding and Alzheimer
Two possible receptors found for prion protein: Lancet
Clue to possible strategy to block mad-cow disease: NY Times
Backgrounder on laminin receptor
Serious caveolin
Caveolin-selected peptide ligands have spaced aromatics
Bax heterodimerizes with Bcl-2
Bax can convert Bcl-2 into activator of apoptosis
Bcl-2 and conserved dimerization domains
Bcl-2, apoptosis, and oxidation state
Bcl-2 conservation of function in mammalian, avian, and viral homologs
Functional domains in Bcl-2
Bcl-2 and nitric acid protection
ER and prion topology: the plot thickens
V. Lingappa offers further clarification
Press release: Prion disease mechanism found
Commentary: prions and the endoplasmic reticulum
Highligts of the Science article
Microglial signal transduction of amyloidogenic fragments
Prion cell biology
Alterations in Potassium Currents May Trigger Neurodegeneration
Astrocyte expression of prion protein
Macrophages transport prions to CNS -- Dealler
TSE persistence: reservoir or replication?
Table with mouse-to-hamster persistence data
How do prion proteins get to the brain?
Promoter up-mutations in amyloid 'chaparone' triple Alzheimer risk
Clusterin: an extracellular version of heat-shock protein
Hsp 104 at Genbank, SwissProt, Blast, and Proteome
Article highlights
Mammalian homologues of Hsp 104
GroEL structure/function and human homologes
Yeast chaperone protein and mad cow disease
Earlier Hsp 104 papers,
Heat Shock Proteins
Antagonistic interactions between yeast chaperones Hsp104 and Hsp70 in prion curing
Chaperone review
Controversy over HSP binding to prion 180-2I study
Yeast Prions and HSP 104

Species barriers, strain types, cell biology

Preclinical BSE: sheep blood shown infectious
Human blood Prpc: reality check on rodents
The risk of blood-borne CJD
Age-related expression of prion protein in human peripheral blood leukocytes
Impaired prion replication in mice lacking follicular dendritic cells
PrPC in peripheral nervous system: neuroinvasion
Hay mites at it again
Peyer's patch prion distribution: enhancing IHC sensitivity
Species-barrier prion replication in apparently resistant species.
Clarification of cross-species in vitro barriers
Application to the species barrier
Prion dimers and the species barrier
What could account for a species barrier.
Phylogenetic distance?
Quasi homo-oligomer interactions?
Natural selection?
Experimental selective bias?
Species barrier references
Definition of strain type 4t
Strain-dependent differences in beta-sheet conformations of rogue prion
Glycosylation differences by brain region
38k form responsible for rapid anterograde axonal transport
Glycosylation differences between the normal and pathogenic prion
Glycotype analysis in familial and sporadic CJD patients
Are strain types a new idea?
Prion glycoform varies by brain region
Definition of strain type 4t
Strain-dependent differences in beta-sheet conformations of rogue prion
3 new PNAS articles on prion seed capping and strain interference
Strain interference in CJD
Conformational basis for prion strains
Highlights of Bruce strain-typing
Highlights of Collinge strain-typing
Eight prion strains have PrPSc molecules with different conformations
Encipherment and propagation of strains
Prion Strain Problem: overview
Strain back-tracking 101
Rescuing knockout mice symptoms with normal prion
Apoptotic cells found in CJD
PrP-dependent association of prions with splenic but not circulating lymphocytes
Peripheral neuron infection varies
Microglial signal transduction of amyloidogenic fragments

Normal prion function

Signal transduction normal role proposed
Enzymatic function found?
Normal prion protein: activity like superoxide dismutase.
Prion Normal Role
Prion gene good for something after all?
Knockouts vs knockouts: is the prion gene essential after all?
Can loss-of-function prion-related diseases exist?
Prion protein expression, superoxide dismutase activity, and iNOS
Active site of SOD3 and regulation of NOS
Prions: nitric oxide, superoxide, and peroxynitrite
Impaired motor coordination in mice lacking prion protein
Cycling in early endosomes
Homology of prion globular domain to signal peptidases?
Signal peptidases are not homologous to prion protein:-- review
Detection of PrpSc by multi-spectral ultraviolet fluorescence
Lethal partial knockout found
Increased calcium in microglia activated by prion
Significant prion titres found outside CNS in clinically normal hamsters
Normal prion function: clue from adjacent genes?
Amino terminus of prion inessential

Sporadic CJD

Surgery increases risk of sporadic CJD
300 cases of sporadic CJD classified
Sporadic fatal insomnia? -- making lemonade out of a misdiagnosis
CJD in 21-year-old in Poland called sporadic
Husband-wife CJD
CJD In Austria and Europe
E219K never occurs in sporadic CJD
Both prion allotypes accumulate in sporadic and Val 21O lle CJD
Protease alleles don't affect sporadic CJD
Prion gene intron alleles and diagnosis of sporadic CJD
CJD stats from Germany
Italian man and pet cat: article review
Belgium claims case of sporadic BSE
Risk of sporadic CJD with codon129 allele
Alpha 1 anti-chymotrypsin polymorphisms in sporadic CJD
Both prion allotypes accumulates in sporadic and val 210 ile?
Most Parkinson's cases sporadic
CJD special issue of Canada Med Assoc J


MRI: specific diagnosis of nvCJD?
Risk of nvCJD in Blood: DNV assessment
90 cases of nvCJD predicted at end of 1998
CJD Surveillance Unit annual report
nvCJD and bovine pituitary growth hormone
90 cases of nvCJD predicted at end of 1998
Florid plaques unique to nvCJD?
Deaths from dementia may hide cases of new variant CJD
Mid-year nvCJD statistics
nvCJD from mononucleosis and tonsilectomies?
Irish nvCJD concern
Estimating the extent of the nvCJD epidemic
Are florid plaques enough?
Tonsil test warrants larger survery
No prion disease in 33 UK haemophilic patients by 1995
Donor pool size and the risk of blood-borne CJD
Almond and Pattison on Human BSE
Smoking gun
Three new CJD articles in 25 Apr 98 Lancet
nvCJD In Ireland?
Collinge on risk to British population
Strain typing of UK farmers with CJD
Appendix provides opportunity
Therapy for nvCJD
Bone marrow: bad news in Blood
New study: Isle of the Damned?
Lancet CJD epidemiology study
Prion infectious titre during incubation of symptoms
Aguzzi et al.: B lymphocytes and neuroinvasion
comentary by Paul Brown
commentary by Gretchen Vogel
B cells: selected abstracts
PrP expression in B lymphocytes

Iatrogenic CJD

Iatrogenic CJD: the ultimate review article
Leucodepletion: does leucocyte fragmentation make matters worse?
Infectivity survives boiling in SDS
Making more amyloid: common diabetic drug
No prion disease in 33 UK haemophilic patients by 1995
Iatrogenic disease: all-time record claimed by hepatitus C
Dermal prions: enough to make your skin crawl
Risk of prion disease transmission from ocular donor tissue transplantation.
Monitoring plasma processing steps
Limited detection of sternal bone marrow infectivity
Detection of prions in collagen and gelatin
Plasma fractionation processes and causative agents of TSE
Cautionary new CJD epidemiology: meat consumption, glaucoma tonometry
Repeated surgery -- enhanced risk for sporadic CJD?
Risk of prion disease transmission from ocular donor tissue transplantation
What the dentist needs to know about TSEs
Contaminated lots implicated in French human growth hormone recipients
New route of exposure: carotid dura mater embolization
Further transmission of CJD via corneal transplant
Cleaning CJD-Contaminated Instruments
Infectivity in blood components


Diagnostics: tonsils, third eyelids, and plasminogen
Preclinical diagnosis of scrapie by IHC: moving towards validation
Structure-based drug design
Threshold survival of scrapie after ashing at 600ÉC
Sheep: biopsy of the mandibular gland better than tonsil?
14-3-3 test now offered in Belgium
Tonsil test warrants larger survery
Monoclonal antibodies in human prion disease
How to improve the clinical diagnosis of CJD
New test for early diagnosis of CJD
Paraffin-embedded tissue blot detects prp(sc) early on
Leptomeningeal melanoma and CJD in a patient with chronic lymphocytic leukaemia.
Argyrophilic grains in late-onset Creutzfeldt-Jakob diseased brain.
Bovine or ovine origin for florid plaque dura mater CJD?
Brain injury does not modify TSE
Monoclonal antibody philosophy
Novel approach to monoclonal antibodies
Comparative monoclonal and polyclonal antibodies
Optimizing prion immunohistochemistry
Novel epitope for specific detection of exogenous prion protein
Carnoy's fixative improves Prp-res immunohistochemistry
The psychiatric manifestation of CJD and mis-diagnosis
Human rabies called CJD in Montana and Washington
1000 brain dementia study: half of CJD cases missed
Dealler to lead blood monoclonal effort
Protein 14-3-3
Increase of neuron-specific enolase in patients with CJD
14-3-3 suffices for CJD diagnosis, neuron-specific enolase, S-100 not needed
NEJM 14-3-3 discussion
Current diagnostic criteria for CJD
Prionics test: sensitivity and specificity
Why is 14-3-3 protein found in new CJD test?
Unknown group patents diagnostic method
14-3-3 test now offered in Belgium
Diagnostics news
Is beef ban followed? Sensitive test for bovine mDNA
S100 Blood check may give simpler CJD diagnosis
Can TSEs be diagnosed from urine?
Researchers closer to test for mad cow disease
Detecting BSE cattle with CSF test
Japan researchers make mad cow breakthrough
Capillary isoelectric focusing of the scrapie prion protein
Detection of scrapie at 4 months via tonsil biopsy
Pullman scrapie test
Interview with researcher K.I. O'Rourke
New Scientist account


Follow-up on heme, amphotericin therapy
Protection of personnel and environment against CJD in pathology laboratories.
Congo red: a universal therapy for prionic diseases?
Follow-up on heme compound therapy
Elimination of prions by branched polyamines and implications for therapeutics
Pentosan binding to mature prion protein
Antibody treatment of Alzheimer amyloid
Heparan sulfate-binding site on Apo-serum amyloid A and therapeutic intervention
Effectiveness of polyene antibiotics in treatment of TSE
Prophylactic potential of pentosan polysulphate in TSEs
Dapsone to delay symptoms in Creutzfeldt-Jakob disease
Prophylactic potential of pentosan polysulphate in TSEs
Dealler suggests pentosan might halt the spread of CJD
Pentosan: earlier studies
CJD Therapy with anti-sense prion mRNA?
Graphic of therapy model
DMSO: does it dissociate amyloid fibrils?
Anthracycline slows prion disease in Syrian hamsters
Fatal cardiac failure after a single dose of anthracycline
Porphyrin inhibition of rogue prion formation
Early drug treatments
Will discovery of prion receptors lead to therapy: Nature Medicine


Statistical Aspects of BSE and vCJD: Models for Epidemics
Detection of BSE-specific rogue prion by heat and guanidine thiocyanate
Differences in proteinase K resistance of BSE and 87V
BSE: obstacles to testing
Why infect more chimps with BSE (when so many are already infected?)
Experimentally induced BSE did not transmit via goat embryos.
Similarities between BSE and an experimental isolate of natural scrapie, CH1641.
Similarities between BSE and an isolate of natural scrapie, CH1641
Collected BSE origin hypotheses
Why not more genetics of cattle?
Known cow polymorphisms don't affect course of BSE
Predictions of BSE-causing mutations from CpG effect
Similarities between BSE and an experimental isolate of natural scrapie, CH1641
Similarities between BSE and an isolate of natural scrapie, CH1641
Collinge method to screen all sheep for BSE
Screening of sheep for BSE: further details
Molecular screening of sheep for BSE
Genetic factors affecting BSE and scrapie susceptibility
BSE and Prions: Uncertainties About the Agent
BSE and Prions: Uncertainties About the Article
Longitudinal trends in the UK BSE epizootic
Bad news on BSE fed to primates
Resistant animals could carry BSE
Maternal Transmission and milk production drop as a sign of BSE
Can US researchers legally possess BSE slides?
Dealler: BSE Research
Solvent extraction as an adjunct to rendering


Sheep reference alleles: 14 with K176N
Scrapie: in vitro conversion of nine natural variants of PrP
Fly larvae and pupae as vectors for scrapie
Transmission of 3 mouse-adapted scrapie strains to murine neuroblastoma cell lines
Could a bee sting cause scrapie? A near miss 29.2 MY ago
Scrapie found throughout GI tract
Population dynamics of scrapie in a sheep flock
Prion polymorphisms in sheep with natural scrapie in Norway
High risk scrapie alleles found in New Zealand
30% scrapie in a closed Romanov flock
Sheep: more in vitro prion conversion
Is scrapie a genetic disease? New Zealand sheep have suspect alleles
Is scrapie solely a genetic disease?
Steering committee on sheep,goats, and monitoring
Role for B cells in neuroinvasive scrapie
Scrapie in India: louping ill vaccine strikes again
Scrapie and knockout mice: abnormal localization and activity of nNOS.
Sensitivity of the Western blot: detection of PrPres in natural sheep scrapie
Genetic factors affecting BSE and scrapie susceptibility
3,000 sheep survey completed (but not released)
Sheep allele update
Hazardous Haplotypes in sheep
High risk sheep alleles for scrapie in Scotland, US
Control of scrapie eventually possible?
Breeding out scrapie -- will it work?
Dot blot: rapid sheep polymorphisms?
Survival time of sheep with scrapie depends on prion genotype
N. Hunter review of scrapie
3,000 sheep survey completed (but not released)
Sensitivity of the Western blot: detection of PrPres in natural sheep scrapie
Antibodies distinguishing sheep alleles
Sheep ApoE alleles: no connection to scrapie
Vertical Transmission
Reported scrapie in US: 1,117 cases (1947-1992)
Australia's scrapie program
High risk sheep alleles found in New Zealand/Australia
New Zealand, Australia scramble to squelch rumors
Ovine scrapie susceptibility in US
Prion gene from African wild goat
Review of mad goat disease
Sicilian goats with scrapie


R.F.Marsh: collected papers
Lots of UK dogs with TSE?
Vacuolar Neuronal Degeneration In Rottweilers
Squirrels: a natural reservoir for TSEs?
Xenotransplantation of porcine organs into man
Neurological disorder, unknown, ibex and chamois
Neuronal vacuolation in raccoons


Zoo Prion Disease: review of scientific literature
Database of 82 zoo animals with prion disease
Switcheroo -- mysteries at the MAFF home page
More cheetah TSE 9 years after 'ban'
The case of the missing pumas, tigers, ocelots, and lion
How many house cats really had TSE?
The Bristol Zoo tigers: 1970-77
Horizontal transmission demonstrated in kudu
Eland, nyala, bison, ankole, arabianory, and scimitar-horned oryx
French primate disaster
Primate BSE quarantine recommendations
TSEs in primates: brief literature survey
Lemur prion sequences predicted
Prosimians: which animals are involved so far?
Earlier BSE problems at French, Australian, and Irish zoos
Further blow aimed at surviving primates
Which zoos, which primates, how many animals?
Raccoon encephalopathy resoved: not a TSE
Cerebral amyloid angiopathy in an aged great spotted woodpecker.


Proof of risk of CWD to humans, cattle and sheep
CWD: conversion of cervid PrP into protease-resistant isoforms
Oral transmission and early lymphoid tropism of CWD
CWD: not attributable to 129L allele in elk
CWD: oral transmission to fawns
4 new CWD articles; bighorn prion sequence
Dermal prions: enough to make your skin crawl
Chronic wasting disease round-up: Feb 99
Mule Deer with CWD: 3 new prion alleles reported
Host range of CWD is altered on passage in ferrets
Chronic wasting disease shows TSEs loose in US

Meetings, books, reviews, people

Cambridge Prion Consortium
Preprint servers off and running: BMJ makes NIH look foolish
A little more at Caprion's web site
Verify your contact information
Prion postdoc opening
A French new list: ESB-liste
Medical Progress: CJD and Related TSEs .. review by Gibbs
US SEAC meets June 2, 1999: FDA blood deferals
Lancet editor threatened by Royal Society food lackeys
More from Tubingen meeting of Sept 1999
US, UK oppose gene withholding
BSE advisers admit giving up a purely scientific role
Tubingen meeting of Sept 23, 1999
TSE symposium in Tubingen Germany: 23-25 September 1999
Postdoc available at U Mass Med School
Reuse of angioplasty catheters and risk of CJD: money vs risk
PNAS okays web preprints
Abstracts released 6 days after press release Maff bibliography: making prion protein in Pichia pastoris, etc.
Beyond Medline: searching full-text of many journals for CJD keywords
Am Soc Cell Biology meeting Dec. 12-16, 1998, San Francisco, CA
New web site and latest publications at Anderson's group
TSE regulation in Germany a muddle
Scientists fight for right to withhold data
Blood Safety and Screening Conference 21 Feb 99
Draft Factual Accounts
The genetics of prions--a contradiction in scholarship?
Role of germinal centers in prion disease: meeting 1 Aug 99
Top scientists sign on to biology pre-print server
Bottom scientists fight new Freedom of Information Act
Oxford reins in nvCJD tonsil modeller
Iceland meeting of 22-27 August, 1998
Summer/Fall 1997 Meeting Abstracts
Am Soc Cell Biology meeting Dec. 12-16, 1998, San Francisco, CA
INSERM Meeting?
Society for Neuroscience LA meeting: 10 Nov 98
Portugal meeting: 18 Nov 98
Washington DC meeting: 7-8 Dec 98
Taos NM 3-8 and 9-14th March 1999
CJD Voice hosts meeting 13-15 June 1999
Thermodynamic stability of human prion P102L, D178N, E200K
Molecular classification of prion diseases without proteinase K
Presence of PrPc on epithelial cells of skin and mucous membranes
FFI symposium conclusions
Fourth Annual Blood Safety & Screening Meeting
Warwick meeting news 16 April 1998
Poster sessions at Iceland meeting
Sept 28, 1998 meeting on risk analysis for BSE in the United States
Sept 24 CJD in blood meeting agenda
Blood Safety and Screening Conference 21 Feb 99
The BASES Project:European research project on BSE policy-making
Erice workshop book finally appears
Obtaining obscure symposium proceedings
Jan 1999 new book: "Prions: Molecular and Cellular Biology" D Harris, ed., Horizon Press
Books on BSE
Book Review: Lethal Legacy by Stephen Dealler
Proposal for prion disease nomenclature
Safety tips for anatomic studies of possible CJD
TSE agents: safe working and the prevention of infection
Who/what is Prionics AG?
Hideyo Inouye, Boston prion researcher Intergen BSA and aprotonin validation study
CDC plays blood politics
MAFF answers email research queries
New Law Could Open Up Lab Books
Awarded NIH grants already in public domain, expert says
Top scientists sign on to biology pre-print server
Bottom scientists fight new Freedom of Information Act
NINDS solicitation for prion experiments on primates
Mad Cow appeal: Researchers need brain and spinal-tap samples
London to get CJD research centre
Richard Bessen at Creighton funded by $738,000 federal grants
Herbert Budka and research in Austria
New web site and Latest publications at Anderson's group
Draft Factual Accounts
Aguzzi/Weissmann on prion research frontiers
Prion research: the next frontiers
Recent BSE-CJD Papers
Heynkes' annotated TSE citation database up to 4687
Medical Progress: CJD and Related TSEs .. review by Gibbs
Prion journals
Upcoming Articles of interest in JBC
Best online site for CJD abstracts?
Recent journal papers
Beyond Medline: searching full-text of many journals for CJD keywords

Historical Aspects

Alzheimer's very first case
1920's Alzheimer's case: diagnosis confirmed
Prion creation theory myth checks out -- sort of
Griffith -- the other papers
Key 1967 paper of Alper et al
Earliest papers of Prusiner
Manuelidis: flogging a dead virino?
R.F.Marsh: collected papers
Kuru and nvCJD
Phenotype-genotype studies in kuru
Kuru -- What really happened?
French vache folle of 1883: more work needed
Vets say it was just Aujeszky's disease (pseudo-rabies)
Detwiler on differential diagnosis of Aujeszky's disease

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