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Record 1,  Textual support,  English

Record number: 1, Textual support number: 1 DEF

A distinct isolate of the infectious agent responsible for the transmissible spongiform encephalopathies that exhibits distinct and stable properties of infection(specific incubation time, distribution of vacuolar lesions and pattern of PrPsc accumulation) independently of the host-genetic context.  2, record 1, English, - prion%20strain

Record 1,  Textual support,  French

Record number: 1, Textual support number: 1 DEF

Isolat de l'agent infectieux des encéphalopathies spongiformes transmissibles gardant des caractères très spécifiques d'infectiosité (temps de latence, évolution de la maladie) indépendamment du contexte génétique de l'hôte.  2, record 1, French, - souche%20de%20prion

Record 1,  Textual support,  Spanish

Record 2,  Textual support,  English

Record number: 2, Textual support number: 1 DEF

A host-encoded siaglycoprotein which is found in all mammals examined to date.  1, record 2, English, - prion%20protein

Record number: 2, Textual support number: 1 OBS

It occurs in two forms at least : the cellular, normal form(PrPc) and the disease-causing abnormal form(PrPsc).  1, record 2, English, - prion%20protein

Record 2,  Textual support,  French

Record number: 2, Textual support number: 1 DEF

Sialoglycoprotéine codée à partir d'un gène cellulaire simple identifié chez tous les mammifères étudiés.  1, record 2, French, - prot%C3%A9ine%20prion

Record number: 2, Textual support number: 1 OBS

Elle existe sous deux isoformes : une forme normale, non infectante appelée PrPc (pour cellulaire) et une forme anormale appelée PrPres (pour résistante à la protéinase K).  1, record 2, French, - prot%C3%A9ine%20prion

Record 2,  Textual support,  Spanish

Record number: 2, Textual support number: 1 CONT

Existe una proteína prión normal presente en las membranas de las células nerviosas, codificada por un gen celular localizado en el cromosoma humano 20.  1, record 2, Spanish, - prote%C3%ADna%20pri%C3%B3n

Record 3,  Textual support,  English

Record number: 3, Textual support number: 1 DEF

Hypothetical model for the existence of various prion strains postulating that PrPsc carries a modification, for example carbohydrate residues, which varies from strain to strain and targets a particular subset of cells. These cells would impart the same modification to the newly formed PrPsc molecules.  2, record 3, English, - targeting%20hypothesis

Record 3,  Textual support,  French

Record 3,  Textual support,  Spanish

Record 4,  Textual support,  English

Record number: 4, Textual support number: 1 DEF

Hypothetical model for the existence of various prion strains postulating that each strain is associated with a different conformation of PrPsc and each of them can convert the PrPc of its host into a likeness of itself.  2, record 4, English, - conformational%20hypothesis

Record 4,  Textual support,  French

Record 4,  Textual support,  Spanish

Record 5,  Textual support,  English

Record number: 5, Textual support number: 1 DEF

Sedimented fibril, seen under the electron microscope, which is an aggregate of the proteinase-resistant isoform(PrPsc) and a pathological hallmark of all transmissible spongiform encephalopathies.  1, record 5, English, - scrapie%20associated%20fibril

Record 5,  Textual support,  French

Record number: 5, Textual support number: 1 DEF

Structure fibrillaire torsadée ou bâtonnet présent dans le cerveau d'un sujet atteint d'une encéphalopathie spongiforme transmissible et observée au microscope électronique après ultracentrifugation de la PrPres.  1, record 5, French, - scrapie%20associated%20fibril

Record 5,  Textual support,  Spanish

Record 6,  Textual support,  English

Record number: 6, Textual support number: 1 DEF

Hypothetical model which holds that the infectious agent(the holoprion) responsible for the transmissible spongiform encephalopathies consists of the association of a nucleic acid(the coprion) and PrPsc(the apoprion).  2, record 6, English, - unified%20theory

Record 6,  Textual support,  French

Record number: 6, Textual support number: 1 DEF

Hypothèse selon laquelle l'agent responsable (l'holoprion) des encéphalopathies spongiformes transmissibles, serait l'association d'un petit acide nucléique (le coprion) avec la PrPres (l'apoprion).  2, record 6, French, - th%C3%A9orie%20unifi%C3%A9e

Record 6,  Textual support,  Spanish

Record 7,  Textual support,  English

Record number: 7, Textual support number: 1 DEF

The abnormal form of the mature Prnp gene product found in tissue of transmissible spongiform encephalopathy sufferers, operationally defined as being partly resistant to digestion by proteinase K under standardized conditions.  3, record 7, English, - PrPsc

Record number: 7, Textual support number: 1 OBS

PrPsc stands for the abnormal disease-causing prion protein where the 'sc' refers to scrapie and PrP-res to the resistive prion protein or residue.  2, record 7, English, - PrPsc

Record 7,  Textual support,  French

Record number: 7, Textual support number: 1 DEF

Forme anormale et insoluble de la protéine prion (PrP) présente dans les tissus atteints d'une encéphalopathie spongiforme transmissible, largement résistante au processus naturel de dégradation des protéines et présentant un aspect en fibrilles ou bâtonnets.  2, record 7, French, - PrPres

Record number: 7, Textual support number: 1 OBS

Elle a été baptisée PrPres pour sa résistance partielle à la protéinase ou encore PrPsc pour scrapie.  2, record 7, French, - PrPres

Record 7,  Textual support,  Spanish

Record 8,  Textual support,  English

Record number: 8, Textual support number: 1 DEF

Macromolecule which might function as a molecular chaperone in the refolding of PrPc into PrPsc.  1, record 8, English, - protein%20X

Record 8,  Textual support,  French

Record number: 8, Textual support number: 1 DEF

Macromolécule hypothétique, associée à la protéine prion, nécessaire à la transmission des symptômes neurologiques ainsi qu'à la conversion de la PrPc en son isoforme pathogène.  2, record 8, French, - prot%C3%A9ine%20X

Record 8,  Textual support,  Spanish

Record 9,  Textual support,  English

Record number: 9, Textual support number: 1 DEF

Within the framework of the protein only hypothesis, it is a partially unfolded monomer which is an intermediate in the formation of PrPsc.  1, record 9, English, - PrP%5Bstar%5D

Record number: 9, Textual support number: 1 OBS

PrP[star]: the word "star" replaces the symbol "star", which can not be reproduced because of technical constraints.  2, record 9, English, - PrP%5Bstar%5D

Record 9,  Textual support,  French

Record number: 9, Textual support number: 1 DEF

Protéine hypothétique intermédiaire dans la conversion de la forme normale PrPc en PrPres due à l'interaction d'un facteur X et de la forme cellulaire du prion.  1, record 9, French, - PrP%5Bast%C3%A9risque%5D

Record number: 9, Textual support number: 1 OBS

PrP[astérisque] : En raison de contraintes informatiques, il a été impossible de reproduire le symbole «astérisque».  2, record 9, French, - PrP%5Bast%C3%A9risque%5D

Record 9,  Textual support,  Spanish

Record 10,  Textual support,  English

Record number: 10, Textual support number: 1 CONT

Scrapie, the prototype of a group of diseases which have the unique property of being both hereditary and infectious, is also exceptional in that it fails to evoke an immune response. Purification of crude scrapie preparations showed a strong association of infectivity with a membrane protein (’PrPSC’); but a protein with the same amino acid sequence (’PrPC’) was subsequently also found in normal mammalian nervous tissue. It is postulated by some investigators that ’PrPSC’ is itself the infectious agent, or the most important part thereof, but in papers making that proposal immunological aspects have not been addressed. Experimental evidence supporting the hypothesis of a membrane fragment as agent has likewise lately not been taken into account. A modified form of the membrane hypothesis could account for immunological as well as genetic aspects of these diseases.  1, record 10, English, - PrPSC

Record 10,  Textual support,  French

Record number: 10, Textual support number: 1 CONT

Rappelons brièvement les connaissances acquises sur ces deux isoformes de la PrP (protéine du prion). On l'a vu, la protéine normale présente chez l'individu sain est facilement dégradée par les protéases. Elle a été appelée PrPc (pour cellaire) ou PrPsen (pour sensible aux protéases), afin de distinguer la forme anormale appelée PrPsc dans le cas de la tremblante (sc pour scrapie) ou PrPes pour résistante.  1, record 10, French, - PrP%20de%20la%20tremblante

Record number: 10, Textual support number: 1 OBS

protéine du prion pathologique  3, record 10, French, - PrP%20de%20la%20tremblante

Record 10,  Textual support,  Spanish

Record 11,  Textual support,  English

Record number: 11, Textual support number: 1 CONT

Prions mediate the pathogenesis of certain neurodegenerative diseases, including bovine spongiform encephalopathy of cattle and Creutzfeldt-Jakob disease of humans. The prion particle consists mainly-if not entirely-of PrPSc, a posttranslationally modified isoform of the cellular host-encoded prion protein(PrPc). It has been suggested that additional cellular factors might be involved in the physiological function of PrPc and in the propagation of PrPSc.  1, record 11, English, - cellular%20host%2Dencoded%20prion%20protein

Record 11,  Textual support,  French

Record number: 11, Textual support number: 1 CONT

Rappelons brièvement les connaissances acquises sur ces deux isoformes de la PRP (protéine du prion). On l'a vu, la protéine normale présente chez l'individu sain est facilement dégradée par les protéases. Elle a été appelée PrPc (pour cellulaire) ou PrPsen (pour sensible aux protéases), afin de distinguer la forme anormale appelée PrPsc dans de la cas de la tremblante (sc pour scrapie) ou PrPes pour résistante.  1, record 11, French, - PrP%20cellulaire

Record number: 11, Textual support number: 1 OBS

Protéine du prion présent chez l'individu sain.  3, record 11, French, - PrP%20cellulaire

Record 11,  Textual support,  Spanish