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PrPsc [2 records]

Filter results by subject field Alphabetical list of terms

Filter results by subject field

Alphabetical list of terms

Record 1 2011-05-09

English

Subject field(s)
  • Viral Diseases
  • Genetics
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.

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.

French

Domaine(s)
  • Maladies virales
  • Génétique
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.

OBS

Elle a été baptisée PrPres pour sa résistance partielle à la protéinase ou encore PrPsc pour scrapie.

Spanish

Campo(s) temático(s)
  • Enfermedades víricas
  • Genética
Save record 1

Record 2 1999-06-16

English

Subject field(s)
  • Biochemistry
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.

French

Domaine(s)
  • Biochimie
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.

OBS

protéine du prion pathologique

Spanish

Save record 2

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