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Propagation of PrP(Sc) in mice reveals impact of aggregate composition on prion disease pathogenesis.
Chang SC, Hannaoui S, Arifin MI, Huang YH, Tang X, Wille H, Gilch S. Chang SC, et al. Among authors: gilch s. Commun Biol. 2023 Nov 14;6(1):1162. doi: 10.1038/s42003-023-05541-3. Commun Biol. 2023. PMID: 37964018 Free PMC article.
Infectious prions consist of PrP(Sc), a misfolded, aggregation-prone isoform of the host's prion protein. ...To investigate the impact of PrP(Sc) aggregate complexity on prion propagation, biochemical properties, and disease pathogenesis, we fractionated elk …
Infectious prions consist of PrP(Sc), a misfolded, aggregation-prone isoform of the host's prion protein. ...To investigate th …
Genetic modulation of CWD prion propagation in cervid PrP Drosophila.
Thackray AM, McNulty EE, Nalls AV, Cardova A, Tran L, Telling G, Benestad SL, Gilch S, Mathiason CK, Bujdoso R. Thackray AM, et al. Among authors: gilch s. Biochem J. 2023 Oct 11;480(19):1485-1501. doi: 10.1042/BCJ20230247. Biochem J. 2023. PMID: 37747806 Free PMC article.
We have generated Drosophila transgenic for S138 wild type cervid PrP, or the N138 variant associated with resistance to chronic wasting disease. We show that cervid PrP Drosophila accumulate bona fide prion infectivity after exposure to cervid prions. Furthermore, …
We have generated Drosophila transgenic for S138 wild type cervid PrP, or the N138 variant associated with resistance to chronic wasting dis …
Cellulose ether treatment inhibits amyloid beta aggregation, neuroinflammation and cognitive deficits in transgenic mouse model of Alzheimer's disease.
Ali T, Klein AN, McDonald K, Johansson L, Mukherjee PG, Hallbeck M, Doh-Ura K, Schatzl HM, Gilch S. Ali T, et al. Among authors: gilch s. J Neuroinflammation. 2023 Jul 28;20(1):177. doi: 10.1186/s12974-023-02858-y. J Neuroinflammation. 2023. PMID: 37507761 Free PMC article.
Recently, we found that cellulose ether compounds (CEs) have beneficial effects against prion diseases by inhibiting protein misfolding and replication of prions, which share their replication mechanism with Abeta. ...
Recently, we found that cellulose ether compounds (CEs) have beneficial effects against prion diseases by inhibiting protein misfoldi …
Peptide aptamer targeting Aβ-PrP-Fyn axis reduces Alzheimer's disease pathologies in 5XFAD transgenic mouse model.
Ali T, Klein AN, Vu A, Arifin MI, Hannaoui S, Gilch S. Ali T, et al. Among authors: gilch s. Cell Mol Life Sci. 2023 May 7;80(6):139. doi: 10.1007/s00018-023-04785-w. Cell Mol Life Sci. 2023. PMID: 37149826 Free PMC article.
Currently, no effective therapeutics exist for the treatment of incurable neurodegenerative diseases such as Alzheimer's disease (AD). The cellular prion protein (PrP(C)) acts as a high-affinity receptor for amyloid beta oligomers (AbetaO), a main neurotoxic species mediat …
Currently, no effective therapeutics exist for the treatment of incurable neurodegenerative diseases such as Alzheimer's disease (AD). The c …
Heterozygosity for cervid S138N polymorphism results in subclinical CWD in gene-targeted mice and progressive inhibition of prion conversion.
Arifin MI, Kaczmarczyk L, Zeng D, Hannaoui S, Lee C, Chang SC, Mitchell G, McKenzie D, Beekes M, Jackson W, Gilch S. Arifin MI, et al. Among authors: gilch s. Proc Natl Acad Sci U S A. 2023 Apr 11;120(15):e2221060120. doi: 10.1073/pnas.2221060120. Epub 2023 Apr 4. Proc Natl Acad Sci U S A. 2023. PMID: 37014866 Free PMC article.
Prions are proteinaceous infectious particles that replicate by structural conversion of the host-encoded cellular prion protein (PrP(C)), causing fatal neurodegenerative diseases in mammals. Species-specific amino acid substitutions (AAS) arising from single nucleo …
Prions are proteinaceous infectious particles that replicate by structural conversion of the host-encoded cellular prion prote …
New developments in prion disease research.
Gilch S, Schätzl HM. Gilch S, et al. Cell Tissue Res. 2023 Apr;392(1):1-5. doi: 10.1007/s00441-023-03760-y. Cell Tissue Res. 2023. PMID: 36918429 No abstract available.
Loss of small GTPase Rab7 activation in prion infection negatively affects a feedback loop regulating neuronal cholesterol metabolism.
Cherry P, Lu L, Shim SY, Ebacher V, Tahir W, Schatzl HM, Hannaoui S, Gilch S. Cherry P, et al. Among authors: gilch s. J Biol Chem. 2023 Feb;299(2):102883. doi: 10.1016/j.jbc.2023.102883. Epub 2023 Jan 7. J Biol Chem. 2023. PMID: 36623732 Free PMC article.
Prion diseases are fatal and infectious neurodegenerative diseases that occur in humans and animals. ...Here, we investigated the molecular events causing an impaired Rab7 membrane attachment and the potential mechanistic link with elevated cholesterol levels in prion …
Prion diseases are fatal and infectious neurodegenerative diseases that occur in humans and animals. ...Here, we investigated the mol …
Chronic wasting disease prions in mule deer interdigital glands.
Ness A, Zeng D, Kuznetsova A, Otero A, Kim C, Saboraki K, Lingle S, Pybus M, Aiken J, Gilch S, McKenzie D. Ness A, et al. Among authors: gilch s. PLoS One. 2022 Oct 3;17(10):e0275375. doi: 10.1371/journal.pone.0275375. eCollection 2022. PLoS One. 2022. PMID: 36190981 Free PMC article.
The disease can be transmitted directly (animal-animal) or indirectly via infectious prions shed into the environment. The precise mechanisms of indirect CWD transmission are unclear but known sources of the infectious prions that contaminate the environment include …
The disease can be transmitted directly (animal-animal) or indirectly via infectious prions shed into the environment. The precise me …
Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD.
Hannaoui S, Zemlyankina I, Chang SC, Arifin MI, Béringue V, McKenzie D, Schatzl HM, Gilch S. Hannaoui S, et al. Among authors: gilch s. Acta Neuropathol. 2022 Oct;144(4):767-784. doi: 10.1007/s00401-022-02482-9. Epub 2022 Aug 22. Acta Neuropathol. 2022. PMID: 35996016 Free PMC article.
Prions cause infectious and fatal neurodegenerative diseases in mammals. ...Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we provide evidence for a zoonotic potential of CWD prions, and its probable signature us …
Prions cause infectious and fatal neurodegenerative diseases in mammals. ...Potential transmission to humans of CWD is a growing conc …
Cholesterol and its reciprocal association with prion infection.
Cashion J, Zhang W, Ali T, Gilch S. Cashion J, et al. Among authors: gilch s. Cell Tissue Res. 2023 Apr;392(1):235-246. doi: 10.1007/s00441-022-03669-y. Epub 2022 Jul 12. Cell Tissue Res. 2023. PMID: 35821439 Review.
Prion diseases are incurable, infectious and fatal neurodegenerative diseases that affect both humans and animals. The pathogenesis of prion disease involves the misfolding of the cellular prion protein, PrP(C), to a disease-causing conformation, PrP(Sc), in …
Prion diseases are incurable, infectious and fatal neurodegenerative diseases that affect both humans and animals. The pathogenesis o …
72 results