Llorens, Franc

[["dc.bibliographiccitation.issue","4"],["dc.bibliographiccitation.journal","Current Neurology and Neuroscience Reports"],["dc.bibliographiccitation.volume","17"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Zarranz, Juan-José"],["dc.contributor.author","Fischer, Andre"],["dc.contributor.author","Zerr, Inga"],["dc.contributor.author","Ferrer, Isidro"],["dc.date.accessioned","2018-04-23T11:47:17Z"],["dc.date.available","2018-04-23T11:47:17Z"],["dc.date.issued","2017"],["dc.description.abstract","Purpose of Review Fatal familiar insomnia (FFI) is an autosomal dominant inherited prion disease caused by D178N mutation in the prion protein gene (PRNP D178N) accompanied by the presence of a methionine at the codon 129 polymorphic site on the mutated allele. FFI is characterized by severe sleep disorder, dysautonomia, motor signs and abnormal behaviour together with primary atrophy of selected thalamic nuclei and inferior olives, and expansion to other brain regions with disease progression. This article reviews recent research on the clinical and molecular aspects of the disease. Recent Findings New clinical and biomarker tools have been implemented in order to assist in the diagnosis of the disease. In addition, the generation of mouse models, the availability of ‘omics’ data in brain tissue and the use of new seeding techniques shed light on the molecular events in FFI pathogenesis. Biochemical studies in human samples also reveal that neuropathological alterations in vulnerable brain regions underlie severe impairment in key cellular processes such as mitochondrial and protein synthesis machinery. Summary Although the development of a therapy is still a major challenge, recent findings represent a step toward understanding of the clinical and molecular aspects of FFI."],["dc.identifier.doi","10.1007/s11910-017-0743-0"],["dc.identifier.gro","3142200"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/13320"],["dc.language.iso","en"],["dc.notes.intern","lifescience updates Crossref Import"],["dc.notes.status","final"],["dc.relation.issn","1528-4042"],["dc.title","Fatal Familial Insomnia: Clinical Aspects and Molecular Alterations"],["dc.type","journal_article"],["dc.type.internalPublication","unknown"],["dc.type.peerReviewed","no"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","257"],["dc.bibliographiccitation.lastpage","263"],["dc.bibliographiccitation.seriesnr","1779"],["dc.contributor.author","Schmitz, Matthias"],["dc.contributor.author","Candelise, Niccolò"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Zerr, Inga"],["dc.contributor.editor","Sigurdsson, Einar M."],["dc.contributor.editor","Calero, Miguel"],["dc.contributor.editor","Gasset, María"],["dc.date.accessioned","2021-06-02T10:44:26Z"],["dc.date.available","2021-06-02T10:44:26Z"],["dc.date.issued","2018"],["dc.identifier.doi","10.1007/978-1-4939-7816-8_16"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/87035"],["dc.notes.intern","DOI-Import GROB-425"],["dc.publisher","Springer New York"],["dc.publisher.place","New York, NY"],["dc.relation.crisseries","Methods in Molecular Biology"],["dc.relation.eisbn","978-1-4939-7816-8"],["dc.relation.isbn","978-1-4939-7815-1"],["dc.relation.ispartof","Methods in Molecular Biology"],["dc.relation.ispartof","Amyloid Proteins"],["dc.relation.ispartofseries","Methods in Molecular Biology; 1779"],["dc.title","Amplification and Detection of Minuscule Amounts of Misfolded Prion Protein by Using the Real-Time Quaking-Induced Conversion"],["dc.type","book_chapter"],["dc.type.internalPublication","yes"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","2189"],["dc.bibliographiccitation.issue","4"],["dc.bibliographiccitation.journal","Molecular Neurobiology"],["dc.bibliographiccitation.lastpage","2199"],["dc.bibliographiccitation.volume","53"],["dc.contributor.author","Schmitz, Matthias"],["dc.contributor.author","Ebert, Elisabeth"],["dc.contributor.author","Stoeck, Katharina"],["dc.contributor.author","Karch, Andre"],["dc.contributor.author","Collins, Steven J."],["dc.contributor.author","Calero, Miguel"],["dc.contributor.author","Sklaviadis, Theodor"],["dc.contributor.author","Laplanche, Jean-Louis"],["dc.contributor.author","Golanska, Ewa"],["dc.contributor.author","Baldeiras, Ines"],["dc.contributor.author","Satoh, Katsuya"],["dc.contributor.author","Sanchez-Valle, Raquel"],["dc.contributor.author","Ladogana, Anna"],["dc.contributor.author","Skinningsrud, Anders"],["dc.contributor.author","Hammarin, Anna-Lena"],["dc.contributor.author","Mitrova, Eva"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Kim, Yong Sun"],["dc.contributor.author","Green, Alison"],["dc.contributor.author","Zerr, Inga"],["dc.date.accessioned","2018-11-07T10:15:19Z"],["dc.date.available","2018-11-07T10:15:19Z"],["dc.date.issued","2016"],["dc.description.abstract","At present, the testing of 14-3-3 protein in cerebrospinal fluid (CSF) is a standard biomarker test in suspected sporadic Creutzfeldt-Jakob disease (sCJD) diagnosis. Increasing 14-3-3 test referrals in CJD reference laboratories in the last years have led to an urgent need to improve established 14-3-3 test methods. The main result of our study was the validation of a commercially available 14-3-3 ELISA next to the commonly used Western blot method as a high-throughput screening test. Hereby, 14-3-3 protein expression was quantitatively analyzed in CSF of 231 sCJD and 2035 control patients. We obtained excellent sensitivity/specificity values of 88 and 96 % that are comparable to the established Western blot method. Since standard protocols and preanalytical sample handling have become more important in routine diagnostic, we investigated in a further step the reproducibility and stability of 14-3-3 as a biomarker for human prion diseases. Ring trial data from 2009 to 2013 revealed an increase of Fleiss' kappa from 0.51 to 0.68 indicating an improving reliability of 14-3-3 protein detection. The stability of 14-3-3 protein under short-term and long-term storage conditions at various temperatures and after repeated freezing/thawing cycles was confirmed. Contamination of CSF samples with blood appears likely to be an important factor at a concentration of more than 2500 erythrocytes/mu L. Hemolysis of erythrocytes with significant release of 14-3-3 protein started after 2 days at room temperature. We first define clear standards for the sample handling, short- and long-term storage of CSF samples as well as the handling of blood- contaminated samples which may result in artificially elevated CSF levels of 14-3-3."],["dc.identifier.doi","10.1007/s12035-015-9167-5"],["dc.identifier.isi","000373641500011"],["dc.identifier.pmid","25947081"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/40787"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Humana Press Inc"],["dc.relation.issn","1559-1182"],["dc.relation.issn","0893-7648"],["dc.title","Validation of 14-3-3 Protein as a Marker in Sporadic Creutzfeldt-Jakob Disease Diagnostic"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","6298"],["dc.bibliographiccitation.issue","17"],["dc.bibliographiccitation.journal","International Journal of Molecular Sciences"],["dc.bibliographiccitation.volume","21"],["dc.contributor.affiliation","Podlesniy, Petar; \t\t \r\n\t\t Neurobiology Unit, Institut d’Investigacions Biomèdiques de Barcelona (CSIC), 08036 Barcelona, Spain, ppodlesniy@gmail.com\t\t \r\n\t\t Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), 08036 Barcelona, Spain, ppodlesniy@gmail.com"],["dc.contributor.affiliation","Llorens, Franc; \t\t \r\n\t\t Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), 08036 Barcelona, Spain, franc.llorens@gmail.com\t\t \r\n\t\t Bellvitge Biomedical Research Institute (IBIDELL), 08908 L’Hospitalet de Llobregat, Spain, franc.llorens@gmail.com\t\t \r\n\t\t Department of Neurology, Clinical Dementia Center, University Medical School, Georg-August University, 37075 Göttingen, Germany, franc.llorens@gmail.com"],["dc.contributor.affiliation","Puigròs, Margalida; \t\t \r\n\t\t Neurobiology Unit, Institut d’Investigacions Biomèdiques de Barcelona (CSIC), 08036 Barcelona, Spain, puigrosserra.m@gmail.com\t\t \r\n\t\t Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), 08036 Barcelona, Spain, puigrosserra.m@gmail.com"],["dc.contributor.affiliation","Serra, Nuria; \t\t \r\n\t\t Neurobiology Unit, Institut d’Investigacions Biomèdiques de Barcelona (CSIC), 08036 Barcelona, Spain, serranuri@gmail.com\t\t \r\n\t\t Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), 08036 Barcelona, Spain, serranuri@gmail.com"],["dc.contributor.affiliation","Sepúlveda-Falla, Diego; \t\t \r\n\t\t Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, 20251 Hamburg, Germany, d.sepulveda-falla@uke.uni-hamburg.de"],["dc.contributor.affiliation","Schmidt, Christian; \t\t \r\n\t\t Department of Neurology, Clinical Dementia Center, University Medical School, Georg-August University, 37075 Göttingen, Germany, Christian.Schmidt@medizin.uni-goettingen.de\t\t \r\n\t\t German Center for Neurodegenerative Diseases (DZNE), 37075 Göttingen, Germany, Christian.Schmidt@medizin.uni-goettingen.de"],["dc.contributor.affiliation","Hermann, Peter; \t\t \r\n\t\t Department of Neurology, Clinical Dementia Center, University Medical School, Georg-August University, 37075 Göttingen, Germany, peter.hermann@med.uni-goettingen.de\t\t \r\n\t\t German Center for Neurodegenerative Diseases (DZNE), 37075 Göttingen, Germany, peter.hermann@med.uni-goettingen.de"],["dc.contributor.affiliation","Zerr, Inga; \t\t \r\n\t\t Department of Neurology, Clinical Dementia Center, University Medical School, Georg-August University, 37075 Göttingen, Germany, IngaZerr@med.uni-goettingen.de\t\t \r\n\t\t German Center for Neurodegenerative Diseases (DZNE), 37075 Göttingen, Germany, IngaZerr@med.uni-goettingen.de"],["dc.contributor.affiliation","Trullas, Ramon; \t\t \r\n\t\t Neurobiology Unit, Institut d’Investigacions Biomèdiques de Barcelona (CSIC), 08036 Barcelona, Spain, ramon.trullas@iibb.csic.es\t\t \r\n\t\t Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), 08036 Barcelona, Spain, ramon.trullas@iibb.csic.es\t\t \r\n\t\t Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain, ramon.trullas@iibb.csic.es"],["dc.contributor.author","Podlesniy, Petar"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Puigròs, Margalida"],["dc.contributor.author","Serra, Nuria"],["dc.contributor.author","Sepúlveda-Falla, Diego"],["dc.contributor.author","Schmidt, Christian"],["dc.contributor.author","Hermann, Peter"],["dc.contributor.author","Zerr, Inga"],["dc.contributor.author","Trullas, Ramon"],["dc.date.accessioned","2021-04-14T08:32:33Z"],["dc.date.available","2021-04-14T08:32:33Z"],["dc.date.issued","2020"],["dc.date.updated","2022-09-07T00:35:16Z"],["dc.identifier.doi","10.3390/ijms21176298"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/83949"],["dc.language.iso","en"],["dc.notes.intern","DOI Import GROB-399"],["dc.relation.eissn","1422-0067"],["dc.rights","CC BY 4.0"],["dc.rights.uri","https://creativecommons.org/licenses/by/4.0/"],["dc.title","Cerebrospinal Fluid Mitochondrial DNA in Rapid and Slow Progressive Forms of Alzheimer’s Disease"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.subtype","original_ja"],["dc.type.version","published_version"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","1863"],["dc.bibliographiccitation.issue","4"],["dc.bibliographiccitation.journal","Molecular Neurobiology"],["dc.bibliographiccitation.lastpage","1874"],["dc.bibliographiccitation.volume","57"],["dc.contributor.author","Schmitz, Matthias"],["dc.contributor.author","Candelise, Niccolo"],["dc.contributor.author","Kanata, Eirini"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Thüne, Katrin"],["dc.contributor.author","Villar-Piqué, Anna"],["dc.contributor.author","da Silva Correia, Susana Margarida"],["dc.contributor.author","Dafou, Dimitra"],["dc.contributor.author","Sklaviadis, Theodoros"],["dc.contributor.author","Appelhans, Dietmar"],["dc.contributor.author","Zerr, Inga"],["dc.date.accessioned","2020-12-10T14:14:28Z"],["dc.date.available","2020-12-10T14:14:28Z"],["dc.date.issued","2019"],["dc.identifier.doi","10.1007/s12035-019-01837-w"],["dc.identifier.eissn","1559-1182"],["dc.identifier.issn","0893-7648"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/71354"],["dc.language.iso","en"],["dc.notes.intern","DOI Import GROB-354"],["dc.title","Validation of Poly(Propylene Imine) Glycodendrimers Towards Their Anti-prion Conversion Efficiency"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","4138"],["dc.bibliographiccitation.issue","6"],["dc.bibliographiccitation.journal","Molecular Neurobiology"],["dc.bibliographiccitation.lastpage","4149"],["dc.bibliographiccitation.volume","54"],["dc.contributor.author","Schmitz, Matthias"],["dc.contributor.author","Dittmar, Kathrin"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Gelpi, Ellen"],["dc.contributor.author","Ferrer, Isidre"],["dc.contributor.author","Schulz-Schaeffer, Walter J."],["dc.contributor.author","Zerr, Inga"],["dc.date.accessioned","2020-12-10T14:14:25Z"],["dc.date.available","2020-12-10T14:14:25Z"],["dc.date.issued","2016"],["dc.identifier.doi","10.1007/s12035-016-9918-y"],["dc.identifier.eissn","1559-1182"],["dc.identifier.issn","0893-7648"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/71343"],["dc.language.iso","en"],["dc.notes.intern","DOI Import GROB-354"],["dc.title","Hereditary Human Prion Diseases: an Update"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","7793"],["dc.bibliographiccitation.issue","16"],["dc.bibliographiccitation.journal","Proceedings of the National Academy of Sciences"],["dc.bibliographiccitation.lastpage","7798"],["dc.bibliographiccitation.volume","116"],["dc.contributor.author","Vallabh, Sonia M."],["dc.contributor.author","Nobuhara, Chloe K."],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Zerr, Inga"],["dc.contributor.author","Parchi, Piero"],["dc.contributor.author","Capellari, Sabina"],["dc.contributor.author","Kuhn, Eric"],["dc.contributor.author","Klickstein, Jacob"],["dc.contributor.author","Safar, Jiri G."],["dc.contributor.author","Nery, Flavia C."],["dc.contributor.author","Swoboda, Kathryn J."],["dc.contributor.author","Geschwind, Michael D."],["dc.contributor.author","Zetterberg, Henrik"],["dc.contributor.author","Arnold, Steven E."],["dc.contributor.author","Minikel, Eric Vallabh"],["dc.contributor.author","Schreiber, Stuart L."],["dc.date.accessioned","2020-12-10T18:12:53Z"],["dc.date.available","2020-12-10T18:12:53Z"],["dc.date.issued","2019"],["dc.identifier.doi","10.1073/pnas.1901947116"],["dc.identifier.eissn","1091-6490"],["dc.identifier.issn","0027-8424"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/74524"],["dc.language.iso","en"],["dc.notes.intern","DOI Import GROB-354"],["dc.title","Prion protein quantification in human cerebrospinal fluid as a tool for prion disease drug development"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","71"],["dc.bibliographiccitation.issue","1-2"],["dc.bibliographiccitation.journal","Dementia and Geriatric Cognitive Disorders"],["dc.bibliographiccitation.lastpage","80"],["dc.bibliographiccitation.volume","43"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Karch, Andre"],["dc.contributor.author","Golanska, Ewa"],["dc.contributor.author","Schmitz, Matthias"],["dc.contributor.author","Lange, Peter"],["dc.contributor.author","Sikorska, Beata"],["dc.contributor.author","Liberski, Pawel P."],["dc.contributor.author","Zerr, Inga"],["dc.date.accessioned","2018-11-07T10:28:45Z"],["dc.date.available","2018-11-07T10:28:45Z"],["dc.date.issued","2017"],["dc.description.abstract","Background: Several biomarkers have been proposed to discriminate sporadic Creutzfeldt-Jakob disease (sCJD) from other dementias and control cases. However, their clinical accuracy depends on the PRNP codon 129 genotype, leaving it unclear how well established markers behave in untested conditions. Methods: We analyzed 14-3-3, tau, p-tau levels, and the p-tau/ tau ratio in a population sample collected from Polish hospitals including nondementia, dementia, and definite sCJD cases and validated their parameters according to previously established cutoffs. Additionally, the correlation between biomarkers and disease duration as well as the influence of the PRNP129 polymorphism are reported. Results: The tau levels and p-tau/tau ratios differed considerably between sCJD and clinically characterized non-CJD cases (p < 0.001). p-tau was only elevated in sCJD when compared to cases without dementia (p < 0.05). Tau and the p-tau/tau ratio showed a sensitivity of 95 and 100%, respectively, in detecting sCJD cases. A negative correlation between tau levels and disease duration, but not the timing of lumbar puncture was observed. Conclusion: The present findings confirmed the value of the p-tau/tau ratio as a robust sCJD biomarker and suggest a role for tau as prognostic marker. (C) 2017 S. Karger AG, Basel."],["dc.identifier.doi","10.1159/000454802"],["dc.identifier.isi","000395664800007"],["dc.identifier.pmid","28056460"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/43497"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","PUB_WoS_Import"],["dc.publisher","Karger"],["dc.relation.issn","1421-9824"],["dc.relation.issn","1420-8008"],["dc.title","Cerebrospinal Fluid Biomarker-Based Diagnosis of Sporadic Creutzfeldt-Jakob Disease: A Validation Study for Previously Established Cutoffs"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","115"],["dc.bibliographiccitation.lastpage","124"],["dc.contributor.author","Zerr, Inga"],["dc.contributor.author","Zafar, Saima"],["dc.contributor.author","Schmitz, Matthias"],["dc.contributor.author","Llorens, Franc"],["dc.date.accessioned","2021-06-02T10:44:30Z"],["dc.date.available","2021-06-02T10:44:30Z"],["dc.date.issued","2018"],["dc.identifier.doi","10.1016/B978-0-12-804279-3.00008-3"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/87066"],["dc.notes.intern","DOI-Import GROB-425"],["dc.publisher","Elsevier"],["dc.relation.isbn","978-0-12-804279-3"],["dc.relation.ispartof","Cerebrospinal Fluid in Neurologic Disorders"],["dc.title","Cerebrospinal fluid in Creutzfeldt–Jakob disease"],["dc.type","book_chapter"],["dc.type.internalPublication","yes"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","95"],["dc.bibliographiccitation.issue","1"],["dc.bibliographiccitation.journal","Prion"],["dc.bibliographiccitation.lastpage","108"],["dc.bibliographiccitation.volume","14"],["dc.contributor.author","Arora, Amandeep Singh"],["dc.contributor.author","Zafar, Saima"],["dc.contributor.author","Latif, Umair"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Mihm, Sabine"],["dc.contributor.author","Kumar, Prateek"],["dc.contributor.author","Tahir, Waqas"],["dc.contributor.author","Thüne, Katrin"],["dc.contributor.author","Shafiq, Mohsin"],["dc.contributor.author","Schmitz, Matthias"],["dc.contributor.author","Zerr, Inga"],["dc.date.accessioned","2020-12-10T18:15:28Z"],["dc.date.available","2020-12-10T18:15:28Z"],["dc.date.issued","2020"],["dc.identifier.doi","10.1080/19336896.2020.1729074"],["dc.identifier.eissn","1933-690X"],["dc.identifier.issn","1933-6896"],["dc.identifier.purl","https://resolver.sub.uni-goettingen.de/purl?gs-1/17401"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/74854"],["dc.language.iso","en"],["dc.notes.intern","DOI Import GROB-354"],["dc.notes.intern","Merged from goescholar"],["dc.rights","Goescholar"],["dc.rights.uri","https://goescholar.uni-goettingen.de/licenses"],["dc.title","The role of cellular prion protein in lipid metabolism in the liver"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.version","published_version"],["dspace.entity.type","Publication"]]