Prion Science Archives: Reverse Chronological Order
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Prion Science Archives: Reverse Chronological Order

Posted 27 Feb 2001:
Doppel nmr structure
Doppel expresion level and onset of ataxia
Strain typing French nvCJD; sheep scrapie strain matches sporadic CJD
Another case of human intergenic splicing: P2Y11 and SSF1
Fourth Alzheimer gene found
Xanthinuria II: another bovine disease that humans get
Dehydrated beta-sheet structure for yeast amyloid
A 7k PrP fragment is the major amyloid protein in GSS A117V
Membrane topology influences N-glycosylation of the prion protein
Joe Gibbs, top neurological researcher, is dead at 76

Posted 22 Dec 2000:
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

Posted 22 Dec 2000:
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
Distant prion relative in Drosophila?
1,525,050 bp human chr 20p contig released
Distinct Dutch 7x repeat CJD
Displaying residue conservation in the prion protein
Doppel bibliography

Posted 30 Nov 2000:
Artefacts: 4-hydroxyproline at Pro 44, polypro II pre-repeat helix
3D modelling of prion glycans and GPI anchor: helix A left for dimer
Warwicker pdb model of domain-swapped dimer
6 new human prion mutations
Another case of intergenic splicing on human chr 1
Prion incubation time QTL in mouse
Iatrogenic CJD: the ultimate review article
PrPC in peripheral nervous system: neuroinvasion
Prion protein colocalizes with dystroglycan complex in brain
Diagnostics: tonsils, third eyelids, and plasminogen

Posted 16 Nov 2000:
Compound and homozygous prion mutations
Predicting missing CJD mutations from CpG effect
Nomenclature and ascertainment issues: recommendations
Bovine and ovine alleles and mutations at CJD-causative sites from CpG effect.
Deletions in prion octapeptide repeat region.
Insertions in prion octapeptide repeat region.
Prion gallery: 3D blow-ups of point mutation environments.
Curated sequence database for prion genes from 80+ species.
Doppel alleles and CpG: ancient tandem prion paralogue.
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 (use chr20:4844409-5855419, prion is D0015)

Posted 15 Oct 2000:
Species-barrier prion replication in apparently resistant species.
Proof of risk of CWD to humans, cattle and sheep
Preclinical BSE: sheep blood shown infectious
Human blood Prpc: reality check on rodents
Preclinical diagnosis of scrapie by IHC: moving towards validation
Linear and conformational prion epitopes
Monomer-dimer equilibrium of native bovine Prpc not seen in recombinant protein
Chaperone BiP binds to mutant prion and mediates proteosome degradation
Ragged N terminus in types of CJD
Signal transduction normal role proposed

Posted 03 Sep 2000:
New doppel neighbor: a tough little gene
5' prion neighbor: ADRA1A (alpha-adrenergic receptor)
Microsatellites in the prion-doppel region
Chr 20 genes with unigene links
Doppel polymorphisms in nvCJD: no apparent connection
2x prion repeats in lemurs
T188A prion mutation in an octogenarian with CJD
Normal function: a lesson from frataxin
Peyer's patch prion distribution: enhancing IHC sensitivity
Prion mRNA in sheep placenta

Posted 23 Jul 2000:
CWD: conversion of cervid PrP into protease-resistant isoforms
Human genome -- still an unfinished mess; ADRA1A is 5' prion neighbor
Cow and sheep doppel genes
Quail, pigeon prion mix-up
3'UTR regulation of mRNA
Copper-induced asparagine 107 deamidation
9x repeat not neurotoxic in cultured cells
Age-related expression of prion protein in human peripheral blood leukocytes
Insulin: the first infectious amyloidosis
Yeast amyloid fibril: second gene influences fibril initiation

Posted 03 Mar 2000:
Doppel protein properties
5 alleles of human doppel
Doppel allele and sequence resourse
CpG hotspots for doppel
Odd new doppel EST
Index to doppel articles
Prion genomics round-up: a human exon 2 EST
Jag2: another prion gene on chromosome 14?
Late onset prion mutations: E196K, V203I, E211Q
Self-assembly of recombinant prion protein of 106 residues

Posted 30 Apr 2000:
The risk of blood-borne CJD
Structure-based drug design
Impaired prion replication in mice lacking follicular dendritic cells
N-methylation - a general strategy to prevent amyloid formation
Inhibition of huntingtin fibrillogenesis by Congo red etc.
Heterologous prion binding, inhibition of conversion, and species barriers
Accumulation of protease-resistant prion protein in 9x insertional mutants
Infectivity of Alzheimer seeding
Amyloid plaques found in aging salmon
Familial Danish dementia
Amyloid channel toxicity: prion, AD, islet amylin, poly-glutamine
Follow-up on heme, amphotericin therapy
Hay mites at it again

Posted 05 Apr 2000:
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.

Threshold survival of scrapie after ashing at 600C
Sheep: biopsy of the mandibular gland better than tonsil?
Prion missing from complete Drosophila genome
Coexistence of Alzheimer and prion amyloid
Blockers for orphan amyloid diseases
Posted 03 Mar 2000:
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

Posted 15 Feb 2000:
Oxford reins in nvCJD tonsil modeller
Fibrillization of human prion 90-231
Optimal toxicity: 106-126
CJD profile in patients homozygous for PRNP E200K.
Disease-finding with GenMap00
A synthetic peptide initiates GSS in transgenic mice
Prion postdoc opening
NEJM 14-3-3 discussion
Current diagnostic criteria for CJD
Monitoring plasma processing steps
Carnoy's fixative improves Prp-res immunohistochemistry
Yeast 'prion' developments

Posted 12 Feb 2000:
Gene within a gene
High prevalence of pathogenic mutations in patients with early-onset dementia
Amyotrophy revisited
Scrapie: in vitro conversion of nine natural variants of PrP
Comparative monoclonal and polyclonal antibodies
Paraffin-embedded tissue blot detects prp(sc) early on
Cambridge Prion Consortium

Posted 10 Feb 2000:
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
Database update log

Posted 07 Feb 2000:
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

Posted 09 Jan 2000:
KIAA0168: nearest neighboring gene 3' of prion protein
Prion gene mapped in horse
Human prion nmr structure
Prion rods contain an inert polysaccharide scaffold.
Increased sensitivity to seizures in mice prion knockouts
38k form responsible for rapid anterograde axonal transport
Preprint servers off and running: BMJ makes NIH look stupid

Posted 05 Jan 2000:
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

Posted 20 Dec 1999:
Fly larvae and pupae as vectors for scrapie
In vitro conditions for prion fibril formation.
Transmissible and genetic prion diseases share a common pathway
Germany: 12 generations of "fatal familial insomnia"
Nmr structure of PrP106-126
Upregulation of lysosomal hydrolases, perforin, and peroxidases in TSE mice
Transmission of 3 mouse-adapted scrapie strains to murine neuroblastoma cell lines
Genes on 187,029 bp of flanking sequence of prion-doppel
New familial encephalopathy with neuroserpin inclusion bodies
Copper potentiation of Alzheimer Abeta neurotoxicity

Posted 29 Nov 1999:
"Alternative splicing" across prion-doppel tandem duplication
Elimination of prions by branched polyamines and implications for therapeutics
Effects of a disrupted disulfide loop
H187R: novel PRNP sequence variant associated with familial encephalopathy.
P101L in mouse alters TSE transmission time
Prominent psychiatric features, early onset in a new insertional mutation
How to improve the clinical diagnosis of CJD
Oral transmission and early lymphoid tropism of CWD
Mutational Analysis of Het-s Prion Analog of Podospora anserina
Interplay of yeast psi prion chaperones

Posted 15 Nov 1999:
9 new prion gene mutations include T188R and P238S
Normal prion protein has an activity like that of superoxide dismutase.
Protocadherin II: cell surface receptor for normal prion
A little more at Caprion's web site
Protocadherin backgrounder
Structure of the protocadherin extra-cellular domain
200-truncated PrP is present in mature sperm
Glycosylation differences between the normal and pathogenic prion
Role of Hsp70-related chaperone in yeast psi prion

Posted 01 Nov 1999:
Verify your contact information
Re-doubling of doppel-prion pair
Six new human doppel transcripts
Flexible region 104-113 studied by epitope-antibody xray
Optimizing prion immunohistochemistry
Sheep reference alleles: 14 with K176N
Peripheral neuron infection varies
Statistical Aspects of BSE and vCJD: Models for Epidemics
Lancet editor threatened by Royal Society food lackeys

Posted 05 Oct 1999:
Doppel disease
CWD: not attributable to 129L allele in elk
CWD: oral transmission to fawns
Detection of BSE-specific rogue prion by heat and guanidine thiocyanate
Raman pH dependence of copper binding to prion octapeptide
Monoclonal antibody philosophy
More from Tubingen meeting of Sept 1999
Prion glycoform varies by brain region
Large new kindred of A117V
Familial dementia: loop insertion polymerization of neuroserpin
Hereditary cardiac amyloidosis

Posted 01 Oct 1999:
Doppel delay of 54 months
Origin of doppel chimeric transcripts
First human doppel allele found: M174T
Doppelcross: cis-trans genetic issues
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
142,634 genes on human chromosome?
US, UK oppose gene withholding

Posted 15 Sep 1999:
Doppel prion upregulated in knockouts
Ancestral origins of E200K -- better chr 20 microsatellites now available
Did a tandem gene doubling create the prion superfamily?
Details on 4th gene: RPS4X pseudogene
Massive parasitism of chromosome 20
Have any genes been missed?
Novel approach to monoclonal antibodies
Glycotype analysis in familial and sporadic CJD patients
Unusual repeat in lemur prion

Posted 03 Sep 1999:
Prnd and Prnp gobbled up by human genome project
Which came first, the prion or the ghost?
Ghost prion promoters
Bridging the gap
Pseudogene prion neighbor: isopentenyl diphosphate:dimethylallyl diphosphate isomerase IPP
Mutant ubiquitinylation tied to brain diseases
Pentosan binding to mature prion protein
French review of FFI: haplotypes and phenotypes
Protection of personnel and environment against CJD in pathology laboratories.
Leptomeningeal melanoma and CJD in a patient with chronic lymphocytic leukaemia.

Posted 29 Aug 1999:
Prion paralogue found adjacent to main gene!
Long and short incubation mouse prion genes compared
Zebrafish RH panel locates prion gene
Could a bee sting cause scrapie? A near miss 29.2 MY ago
Anti-prion 4.5 kb mRNA: a harmless prion pseudogene?
Alternate exon 1 splicing in Holstein
MRI: specific diagnosis of nvCJD?
Cerebral amyloid angiopathy in an aged great spotted woodpecker.
Prion octapeptide: intermolecular copper

Posted 08 Aug 1999:
Enzymatic function found?
Implications of (Tylopoda, (Suidae, (Cetaceae, (chevrotain,...))))
7 new bird prion sequences
4 new CWD articles; bighorn prion sequence
Inhibition of in vitro conversion by 109 to 141
Variants of cross-beta region 106-126
106-126 isoaspartate?
New mutation, H187R, tracks with GSS
Y145 stop: proteasomal degradation and PK resistance
300 cases of sporadic CJD classified
Transmission/induction of another amyloidosis by diet
Leucodepletion: does leucocyte fragmentation make matters worse?
BSE advisers admit giving up a purely scientific role

Posted 20 Jul 1999:
Cautionary new CJD epidemiology: meat consumption, glaucoma tonometry
New human amyloid identified
Tubingen meeting of Sept 23, 1999
Differences in proteinase K resistance of BSE and 87V
Argyrophilic grains in late-onset Creutzfeldt-Jakob diseased brain.
Bovine or ovine origin for florid plaque dura mater CJD?
Glycosylation differences by brain region
The genetics of prions--a contradiction in scholarship?
Prion polyA variation -- does it lead to over-production?
Postdoc available at U Mass Med School

Posted 01 Jul 1999:
Antibody treatment of Alzheimer amyloid
BSE: obstacles to testing
Infectivity survives boiling in SDS
Scrapie found throughout GI tract
27 new mammalian and 9 bird prions: high conservation of flexible region
Circadian regulation of prion mRNA: widespread synchronous rhythm.
Rescuing knockout mice symptoms with normal prion
Apoptotic cells found in CJD
Selective oxidation of prion methionine residues
Equilibrium folding properties of the yeast prion Ure2
Altered phospholipase release of prion mutants
Iatrogenic disease: all-time record claimed by hepatitus C

Posted 18 Jun1999:
Sheep: more in vitro prion conversion
Conversion of normal prion via a localized domain
Protease-resistant, detergent-insoluble prion not necessarily infectious
Deliberate domino iatrogenic amyloidosis
AD amyloid peroxide causes Alzheimer's damage?
Clusterin prevents aggregation of neuropeptide 106-126 in vitro.
Population dynamics of scrapie in a sheep flock
Prion polymorphisms in sheep with natural scrapie in Norway
Monoclonal antibodies in human prion disease
E200K + valine at codon 129 = type 2 protease-resistant prion
Brain injury does not modify TSE

Posted 15 Jun 1999:
Familial Mediterranean Fever: indirect serum amyloid
Friedreich ataxia: non-amyloid intronic polyGAA repeat
Spinobulbar muscular atrophy -- X-linked androgen amyloid?
Oculopharyngeal dystrophy: autosomal dominant poly-alanine expansion
Wernicke encephalopathy (alcoholism) and CJD: chicken or egg?
Familial British dementia: read-thru of stop codon in cerebral amyloid angiopathy
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
Dentatorubralpallidoluysian atrophy: are fibrils congophilic?

Posted 06 Jun 1999:
Molecular model of Alzheimer fibril
Huntingon: seeded fibril nucleation
Fragment-enhancing Alzheimer mutation
Acylphosphatase: can any protein form amyloid protofilaments and fibrils?
Poly-Q domain of the Drosophila GAGA forms amyloid fibre
A30P and A53T amyloid filaments of alpha-synuclein (NAC) in PD
NAC is congophilic, may seed Abeta deposition
Genome-wide screens for polyglutamine disease
Phenotypic heterogeneity of FTDP-17: alternative splicing increases 4 repeat tau
Exposure of cryptic epitopes on transthyretin only in amyloidogenic mutants
BIGH3 -- transmissible congophilic corneal amyloid?
Fibrinogen alpha amyloid: renal transplant 'infected'
Earliest recognition of cross-beta architecture

Posted 04 Jun 1999:
Making more amyloid: common diabetic drug
Two prion-inducing regions of ure2p are nonoverlapping
PrP-dependent association of prions with splenic but not circulating lymphocytes
Raccoon encephalopathy resoved: not a TSE
Copper chelators and strain types 1 and 2
Sporadic fatal insomnia? -- making lemonade out of a misdiagnosis
Reuse of angioplasty catheters and risk of CJD: money vs risk
Is scrapie a genetic disease? New Zealand sheep have suspect alleles
Steering committee on sheep,goats, and monitoring

Posted 25 May 1999:
Analysis of the prion gene: introduction
Feature tables of mammalian prion genes
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

Posted 10 May 1999:
Prion glycan: bittersweet situation
30% scrapie in a closed Romanov flock
TSE symposium in Tubingen Germany: 23-25 September 1999
French E200K cluster
A French new list: ESB-liste
High risk scrapie alleles found in New Zealand
US SEAC meets June 2, 1999: FDA blood deferals
Guinea pig prion sequence: PCR errors claimed
Plasma fractionation processes and causative agents of TSE

Posted 22 Apr 1999:
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

Posted 15 Apr 1999:
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?
Why infect more chimps with BSE (when so many are already infected?)

Posted 10 Apr 1999:
CJD Surveillance Unit annual report
Repeated surgery -- enhanced risk for sporadic CJD?
Cow prion with 7 repeats
Role for B cells in neuroinvasive scrapie
Mini-prion up and running
PNAS okays web preprints
Ancestral origin of E200K: 6 founding mutations in 62 families
Heparan sulfate-binding site on Apo-serum amyloid A and therapeutic intervention
Increase of neuron-specific enolase in patients with CJD
Limited detection of sternal bone marrow infectivity
A systematic search for repeat conformational disorders
Mouse prion glycans determined
Real biological role for yeast prions?
Refinement of hamster nmr

Posted 25 Mar 1999:
Control of disulphides in the endoplasmic reticulum and in vitro
Application of distance geometry to 3D visualization of prion sequence relationships
Improved NMR structure of Syrian hamster prion
Effectiveness of polyene antibiotics in treatment of TSE
Gamma-secretase enahncement of amyloid precursor protein
Exposure of cryptic epitopes on transthyretin only in amyloid
Experimentally induced BSE did not transmit via goat embryos.
Scrapie in India: louping ill vaccine strikes again
Prionics test: sensitivity and specificity
Detection of prions in collagen and gelatin
Role of germinal centers in prion disease: meeting 1 Aug 99
Maff bibliography: making prion protein in Pichia pastoris, etc.
CJD in 21-year-old in Poland called sporadic

Posted 06 Mar 1999:
GG Glenner was right: yeast prions URE3 and Sup35 are congo red birefringent
Structure of SH3 cross beta fibril
Surgery increases risk of sporadic CJD
14-3-3 suffices for CJD diagnosis, neuron-specific enolase, S-100 not needed
Met/met at 129 predisposes to sporadic inclusion-body myositis
Scrapie and knockout mice: abnormal localization and activity of nNOS.
D178N: first Austrian kindred
Laminin binds another amyloid former, serum amyloid A.
Novel epitope for specific detection of exogenous prion protein
Risk of nvCJD in Blood: DNV assessment
TSE regulation in Germany a muddle
Scientists fight for right to withhold data

Posted 02 Feb 1999:
Dermal prions: enough to make your skin crawl
90 cases of nvCJD predicted at end of 1998
Blood Safety and Screening Conference 21 Feb 99
Draft Factual Accounts
Risk of prion disease transmission from ocular donor tissue transplantation.
Microglial signal transduction of amyloidogenic fragments
Prophylactic potential of pentosan polysulphate in TSEs
Most Parkinson's cases sporadic
Protease-resistant prion produced in vitro lacks detectable infectivity.
Similarities between BSE and an experimental isolate of natural scrapie, CH1641.

Posted 16 Jan 1999:
Tonsil test warrants larger survery
Definition of strain type 4t
3,000 sheep survey completed (but not released)
Chicken prion tandem repeats form a stable, protease-resistant domain.
Antagonistic interactions between yeast chaperones Hsp104 and Hsp70 in prion curing.
Making (anti)sense of non-coding sequence conservation
Top scientists sign on to biology pre-print server
Bottom scientists fight new Freedom of Information Act

Posted 09 Jan 1999:
Odd prion allele found in goat
Impaired motor coordination in mice lacking prion protein.
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
14-3-3 test now offered in Belgium
Mini-review: The Yeast Psi+ Prion: Making Sense of Nonsense
Similarities between BSE and an isolate of natural scrapie, CH1641
Ubiquitinated inclusions in prion diseases

Posted 01 Jan 99:
Copper stimulates endocytosis of the prions
Copper chaperones: no known homology to prion protein
Cycling in early endosomes
Strain-dependent differences in beta-sheet conformations of rogue prion
Rapid acquisition of beta-sheet structure in the prion protein prior to multimer formation.
No prion disease in 33 UK haemophilic patients by 1995
Medical Progress: CJD and Related TSEs ... review by Gibbs
Sensitivity of the Western blot: detection of PrPres in natural sheep scrapie
New E200K cluster in Japan
Genetic factors affecting BSE and scrapie susceptibility

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