Loss of functional prion protein: a role in prion disorders?
To understand the normal function of the prion protein (PrP) and its role in prion disorders, several groups have generated mice lacking PrP. Some of these mice develop symptoms associated with prion diseases, but other experimental evidence suggests that the loss of functional PrP is not the instigating factor in these disorders.
Borchelt
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Department of Pathology, The Johns Hopkins University School of Medicine, 720 Rutland Avenue, 558 Ross Building, Baltimore, MD 21205, USA. drbor@welchlink.welch.jhu.edu
Sisodia
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D000818:Animals; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D008822:Mice, Transgenic; D010641:Phenotype; D018621:PrPC Proteins / Q000472:pathogenicity; D018620:PrPSc Proteins / Q000235:genetics / Q000472:pathogenicity; D017096:Prion Diseases / Q000209:etiology*; D011328:Prions / Q000235:genetics / Q000472:pathogenicity*
D018621:PrPC Proteins; D018620:PrPSc Proteins; D011328:Prions