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Phenotypic variability of sporadic human prion disease and its molecular basis: past, present, and future.
Parchi P, Strammiello R, Giese A, Kretzschmar H. Parchi P, et al. Among authors: giese a. Acta Neuropathol. 2011 Jan;121(1):91-112. doi: 10.1007/s00401-010-0779-6. Epub 2010 Nov 24. Acta Neuropathol. 2011. PMID: 21107851 Review.
In comparison to other more common neurodegenerative disorders, prion diseases show a wider range of phenotypic variation and largely transmit to experimental animals, a feature that led to the isolation and characterization of different strains of the transmissible …
In comparison to other more common neurodegenerative disorders, prion diseases show a wider range of phenotypic variation and largely …
Comprehensive neuropathologic analysis of genetic prion disease associated with the E196K mutation in PRNP reveals phenotypic heterogeneity.
Eigenbrod S, Frick P, Giese A, Schelzke G, Zerr I, Kretzschmar HA. Eigenbrod S, et al. Among authors: giese a. J Neuropathol Exp Neurol. 2011 Mar;70(3):192-200. doi: 10.1097/NEN.0b013e31820cd8a4. J Neuropathol Exp Neurol. 2011. PMID: 21293298
We report a comprehensive analysis of 4 patients carrying the rare E196K (GAG→AAG) mutation who presented with clinical features of CJD. ...We report histopathologic and biochemical findings in addition to clinical observations, thus providing a more comprehensive a …
We report a comprehensive analysis of 4 patients carrying the rare E196K (GAG→AAG) mutation who presented with clinical features of C …
Reducing tau aggregates with anle138b delays disease progression in a mouse model of tauopathies.
Wagner J, Krauss S, Shi S, Ryazanov S, Steffen J, Miklitz C, Leonov A, Kleinknecht A, Göricke B, Weishaupt JH, Weckbecker D, Reiner AM, Zinth W, Levin J, Ehninger D, Remy S, Kretzschmar HA, Griesinger C, Giese A, Fuhrmann M. Wagner J, et al. Among authors: giese a. Acta Neuropathol. 2015 Nov;130(5):619-31. doi: 10.1007/s00401-015-1483-3. Epub 2015 Oct 6. Acta Neuropathol. 2015. PMID: 26439832 Free PMC article.
In addition, we found decreased synapse and neuron loss accompanied by a decreased gliosis in the hippocampus. Our results suggest that reducing tau aggregates with anle138b may represent an effective and promising approach for the treatment of human tauopathies....
In addition, we found decreased synapse and neuron loss accompanied by a decreased gliosis in the hippocampus. Our results suggest th …
Quantifying prion disease penetrance using large population control cohorts.
Minikel EV, Vallabh SM, Lek M, Estrada K, Samocha KE, Sathirapongsasuti JF, McLean CY, Tung JY, Yu LP, Gambetti P, Blevins J, Zhang S, Cohen Y, Chen W, Yamada M, Hamaguchi T, Sanjo N, Mizusawa H, Nakamura Y, Kitamoto T, Collins SJ, Boyd A, Will RG, Knight R, Ponto C, Zerr I, Kraus TF, Eigenbrod S, Giese A, Calero M, de Pedro-Cuesta J, Haïk S, Laplanche JL, Bouaziz-Amar E, Brandel JP, Capellari S, Parchi P, Poleggi A, Ladogana A, O'Donnell-Luria AH, Karczewski KJ, Marshall JL, Boehnke M, Laakso M, Mohlke KL, Kähler A, Chambert K, McCarroll S, Sullivan PF, Hultman CM, Purcell SM, Sklar P, van der Lee SJ, Rozemuller A, Jansen C, Hofman A, Kraaij R, van Rooij JG, Ikram MA, Uitterlinden AG, van Duijn CM; Exome Aggregation Consortium (ExAC), Daly MJ, MacArthur DG. Minikel EV, et al. Among authors: giese a. Sci Transl Med. 2016 Jan 20;8(322):322ra9. doi: 10.1126/scitranslmed.aad5169. Sci Transl Med. 2016. PMID: 26791950 Free PMC article.
More than 100,000 genetic variants are reported to cause Mendelian disease in humans, but the penetrance-the probability that a carrier of the purported disease-causing genotype will indeed develop the disease-is generally unknown. ...We also show that truncating variants …
More than 100,000 genetic variants are reported to cause Mendelian disease in humans, but the penetrance-the probability that a carri …
Prion-related peripheral neuropathy in sporadic Creutzfeldt-Jakob disease.
Baiardi S, Redaelli V, Ripellino P, Rossi M, Franceschini A, Moggio M, Sola P, Ladogana A, Fociani P, Magherini A, Capellari S, Giese A, Caughey B, Caroppo P, Parchi P. Baiardi S, et al. Among authors: giese a. J Neurol Neurosurg Psychiatry. 2019 Apr;90(4):424-427. doi: 10.1136/jnnp-2018-319221. Epub 2018 Oct 24. J Neurol Neurosurg Psychiatry. 2019. PMID: 30355606
Nerve biopsy showed a mixed predominantly axonal and demyelinating neuropathy in two sCJDMV2K. Electromyography showed signs of neuropathy in half of the examined VV2-MV2K patients. ...
Nerve biopsy showed a mixed predominantly axonal and demyelinating neuropathy in two sCJDMV2K. Electromyography showed signs of neuro …
Anle138b: a novel oligomer modulator for disease-modifying therapy of neurodegenerative diseases such as prion and Parkinson's disease.
Wagner J, Ryazanov S, Leonov A, Levin J, Shi S, Schmidt F, Prix C, Pan-Montojo F, Bertsch U, Mitteregger-Kretzschmar G, Geissen M, Eiden M, Leidel F, Hirschberger T, Deeg AA, Krauth JJ, Zinth W, Tavan P, Pilger J, Zweckstetter M, Frank T, Bähr M, Weishaupt JH, Uhr M, Urlaub H, Teichmann U, Samwer M, Bötzel K, Groschup M, Kretzschmar H, Griesinger C, Giese A. Wagner J, et al. Among authors: giese a. Acta Neuropathol. 2013 Jun;125(6):795-813. doi: 10.1007/s00401-013-1114-9. Epub 2013 Apr 19. Acta Neuropathol. 2013. PMID: 23604588 Free PMC article.
Here we describe the novel oligomer modulator anle138b [3-(1,3-benzodioxol-5-yl)-5-(3-bromophenyl)-1H-pyrazole], an aggregation inhibitor we developed based on a systematic high-throughput screening campaign combined with medicinal chemistry optimization. ...Our findings i …
Here we describe the novel oligomer modulator anle138b [3-(1,3-benzodioxol-5-yl)-5-(3-bromophenyl)-1H-pyrazole], an aggregation inhibitor we …
Design of anti- and pro-aggregation variants to assess the effects of methionine oxidation in human prion protein.
Wolschner C, Giese A, Kretzschmar HA, Huber R, Moroder L, Budisa N. Wolschner C, et al. Among authors: giese a. Proc Natl Acad Sci U S A. 2009 May 12;106(19):7756-61. doi: 10.1073/pnas.0902688106. Epub 2009 Apr 28. Proc Natl Acad Sci U S A. 2009. PMID: 19416900 Free PMC article.
It has been speculated that methionine (Met) oxidation in PrP(C) may have a special role in this process, but has not been detailed and assigned individually to the 9 Met residues of full-length, recombinant human PrP(C) [rhPrP(C)(23-231)]. ...The experimental results full …
It has been speculated that methionine (Met) oxidation in PrP(C) may have a special role in this process, but has not been detailed a …
Establishing quantitative real-time quaking-induced conversion (qRT-QuIC) for highly sensitive detection and quantification of PrPSc in prion-infected tissues.
Shi S, Mitteregger-Kretzschmar G, Giese A, Kretzschmar HA. Shi S, et al. Among authors: giese a. Acta Neuropathol Commun. 2013 Aug 2;1:44. doi: 10.1186/2051-5960-1-44. Acta Neuropathol Commun. 2013. PMID: 24252329 Free PMC article.
However, as there can also be a fraction of pathological PrP that is digested by PK, we use the term PrP27-30 to denote to the amount of PrPSc that can be detected by immunoblot after PK treatment. qRT-QuIC is based upon the quantitative correlation between the seeded amou …
However, as there can also be a fraction of pathological PrP that is digested by PK, we use the term PrP27-30 to denote to the amount …
Generation of genuine prion infectivity by serial PMCA.
Weber P, Giese A, Piening N, Mitteregger G, Thomzig A, Beekes M, Kretzschmar HA. Weber P, et al. Among authors: giese a. Vet Microbiol. 2007 Aug 31;123(4):346-57. doi: 10.1016/j.vetmic.2007.04.004. Epub 2007 Apr 7. Vet Microbiol. 2007. PMID: 17493773
Analysis of Conformational Stability of Abnormal Prion Protein Aggregates across the Spectrum of Creutzfeldt-Jakob Disease Prions.
Cescatti M, Saverioni D, Capellari S, Tagliavini F, Kitamoto T, Ironside J, Giese A, Parchi P. Cescatti M, et al. Among authors: giese a. J Virol. 2016 Jun 24;90(14):6244-6254. doi: 10.1128/JVI.00144-16. Print 2016 Jul 15. J Virol. 2016. PMID: 27122583 Free PMC article.
In particular, MM1 and VV2, the most prevalent and fast-replicating CJD types, showed stable and highly resistant PrP(Sc) aggregates, whereas VV1, a rare and slowly propagating type, revealed unstable aggregates that easily dissolved at low temperature. ...Furthermore, the …
In particular, MM1 and VV2, the most prevalent and fast-replicating CJD types, showed stable and highly resistant PrP(Sc) aggregates, wherea …
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