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Prions, prionoids and protein misfolding disorders.
Scheckel C, Aguzzi A. Scheckel C, et al. Nat Rev Genet. 2018 Jul;19(7):405-418. doi: 10.1038/s41576-018-0011-4. Nat Rev Genet. 2018. PMID: 29713012 Review.
The term 'prion' was first nominated to express the revolutionary concept that a protein could be infectious. We now know that prions consist of PrP(Sc), the pathological aggregated form of the cellular prion protein PrP(C). ...
The term 'prion' was first nominated to express the revolutionary concept that a protein could be infectious. We now know that prions …
Normal host prion protein necessary for scrapie-induced neurotoxicity.
Brandner S, Isenmann S, Raeber A, Fischer M, Sailer A, Kobayashi Y, Marino S, Weissmann C, Aguzzi A. Brandner S, et al. Nature. 1996 Jan 25;379(6563):339-43. doi: 10.1038/379339a0. Nature. 1996. PMID: 8552188
Accumulation of the prion protein PrPSc, a pathological and protease-resistant isoform of the normal host protein PrPC, is a feature of prion disease such as scrapie. ...
Accumulation of the prion protein PrPSc, a pathological and protease-resistant isoform of the normal host protein PrPC, is a f …
Porphobilinogen deaminase deficiency in mice causes a neuropathy resembling that of human hepatic porphyria.
Lindberg RL, Porcher C, Grandchamp B, Ledermann B, Bürki K, Brandner S, Aguzzi A, Meyer UA. Lindberg RL, et al. Nat Genet. 1996 Feb;12(2):195-9. doi: 10.1038/ng0296-195. Nat Genet. 1996. PMID: 8563760
Acute intermittent porphyria (AIP) is a human disease resulting from a dominantly inherited partial deficiency of the heme biosynthetic enzyme, porphobilinogen deaminase (PBGD). ...
Acute intermittent porphyria (AIP) is a human disease resulting from a dominantly inherited partial deficiency of the heme bio …
Pathogenesis of spongiform encephalopathies: an update.
Aguzzi A. Aguzzi A. Int Arch Allergy Immunol. 1996 Jun;110(2):99-106. doi: 10.1159/000237273. Int Arch Allergy Immunol. 1996. PMID: 8646000 Review.
Sleepless in Bologna: transmission of fatal familial insomnia.
Aguzzi A, Weissmann C. Aguzzi A, et al. Trends Microbiol. 1996 Apr;4(4):129-31; discussion 131-2. doi: 10.1016/0966-842x(96)30012-7. Trends Microbiol. 1996. PMID: 8728604 Review. No abstract available.
Normal host prion protein (PrPC) is required for scrapie spread within the central nervous system.
Brandner S, Raeber A, Sailer A, Blättler T, Fischer M, Weissmann C, Aguzzi A. Brandner S, et al. Proc Natl Acad Sci U S A. 1996 Nov 12;93(23):13148-51. doi: 10.1073/pnas.93.23.13148. Proc Natl Acad Sci U S A. 1996. PMID: 8917559 Free PMC article.
To determine whether PrPC is required for scrapie spread, we inoculated prions intraocularly into Prnp% mice containing a PrP-overexpressing neurograft. ...
To determine whether PrPC is required for scrapie spread, we inoculated prions intraocularly into Prnp% mice containing a PrP-overexp …
The absence of c-fos prevents light-induced apoptotic cell death of photoreceptors in retinal degeneration in vivo.
Hafezi F, Steinbach JP, Marti A, Munz K, Wang ZQ, Wagner EF, Aguzzi A, Remé CE. Hafezi F, et al. Nat Med. 1997 Mar;3(3):346-9. doi: 10.1038/nm0397-346. Nat Med. 1997. PMID: 9055866
In the retina, c-fos is physiologically expressed in a diurnal manner and is inducible by light. We previously observed a light-elicited, dose-dependent apoptotic response in rat photoreceptors. ...Suppl. 36, S638 and Rich, K.A. et al., Invest. Ophthalmol. Vi …
In the retina, c-fos is physiologically expressed in a diurnal manner and is inducible by light. We previously observed a ligh …
Neuro-immune connection in spread of prions in the body?
Aguzzi A. Aguzzi A. Lancet. 1997 Mar 15;349(9054):742-3. doi: 10.1016/S0140-6736(97)22011-8. Lancet. 1997. PMID: 9074567 No abstract available.
Neurotoxicity and neuroinvasiveness of prions.
Aguzzi A, Raeber A, Blättler T, Flechsig E, Klein M, Weissmann C, Brandner S. Aguzzi A, et al. J Neurovirol. 1997 May;3 Suppl 1:S23-4. J Neurovirol. 1997. PMID: 9179785 No abstract available.
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