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Llorens, Franc
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Llorens, Franc
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Llorens, Franc
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Llorens, F.
Llorens Torres, Francesc Josep
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2017Journal Article [["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"]]Details DOI2016Journal Article [["dc.bibliographiccitation.firstpage","95"],["dc.bibliographiccitation.issue","1"],["dc.bibliographiccitation.journal","Brain Pathology"],["dc.bibliographiccitation.lastpage","106"],["dc.bibliographiccitation.volume","27"],["dc.contributor.author","Frau-Méndez, Margalida A."],["dc.contributor.author","Fernández-Vega, Iván"],["dc.contributor.author","Ansoleaga, Belén"],["dc.contributor.author","Blanco Tech, Rosa"],["dc.contributor.author","Carmona Tech, Margarita"],["dc.contributor.author","Antonio del Rio, Jose"],["dc.contributor.author","Zerr, Inga"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","José Zarranz, Juan"],["dc.contributor.author","Ferrer, Isidro"],["dc.date.accessioned","2020-12-10T18:27:04Z"],["dc.date.available","2020-12-10T18:27:04Z"],["dc.date.issued","2016"],["dc.identifier.doi","10.1111/bpa.12408"],["dc.identifier.issn","1015-6305"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/76235"],["dc.language.iso","en"],["dc.notes.intern","DOI Import GROB-354"],["dc.title","Fatal familial insomnia: mitochondrial and protein synthesis machinery decline in the mediodorsal thalamus"],["dc.title.alternative","Fatal Familial Insomnia"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dspace.entity.type","Publication"]]Details DOI2017Journal Article [["dc.bibliographiccitation.artnumber","83"],["dc.bibliographiccitation.issue","1"],["dc.bibliographiccitation.journal","Molecular Neurodegeneration"],["dc.bibliographiccitation.volume","12"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Thüne, Katrin"],["dc.contributor.author","Tahir, Waqas"],["dc.contributor.author","Kanata, Eirini"],["dc.contributor.author","Diaz-Lucena, Daniela"],["dc.contributor.author","Xanthopoulos, Konstantinos"],["dc.contributor.author","Kovatsi, Eleni"],["dc.contributor.author","Pleschka, Catharina"],["dc.contributor.author","Garcia-Esparcia, Paula"],["dc.contributor.author","Schmitz, Matthias"],["dc.contributor.author","Ozbay, Duru"],["dc.contributor.author","Correia, Susana"],["dc.contributor.author","Correia, Ângela"],["dc.contributor.author","Milosevic, Ira"],["dc.contributor.author","Andréoletti, Olivier"],["dc.contributor.author","Fernández-Borges, Natalia"],["dc.contributor.author","Vorberg, Ina M."],["dc.contributor.author","Glatzel, Markus"],["dc.contributor.author","Sklaviadis, Theodoros"],["dc.contributor.author","Torres, Juan Maria"],["dc.contributor.author","Krasemann, Susanne"],["dc.contributor.author","Sánchez-Valle, Raquel"],["dc.contributor.author","Ferrer, Isidro"],["dc.contributor.author","Zerr, Inga"],["dc.date.accessioned","2019-07-09T11:44:59Z"],["dc.date.available","2019-07-09T11:44:59Z"],["dc.date.issued","2017"],["dc.description.abstract","Background YKL-40 (also known as Chitinase 3-like 1) is a glycoprotein produced by inflammatory, cancer and stem cells. Its physiological role is not completely understood but YKL-40 is elevated in the brain and cerebrospinal fluid (CSF) in several neurological and neurodegenerative diseases associated with inflammatory processes. Yet the precise characterization of YKL-40 in dementia cases is missing. Methods In the present study, we comparatively analysed YKL-40 levels in the brain and CSF samples from neurodegenerative dementias of different aetiologies characterized by the presence of cortical pathology and disease-specific neuroinflammatory signatures. Results YKL-40 was normally expressed in fibrillar astrocytes in the white matter. Additionally YKL-40 was highly and widely expressed in reactive protoplasmic cortical and perivascular astrocytes, and fibrillar astrocytes in sporadic Creutzfeldt-Jakob disease (sCJD). Elevated YKL-40 levels were also detected in Alzheimer’s disease (AD) but not in dementia with Lewy bodies (DLB). In AD, YKL-40-positive astrocytes were commonly found in clusters, often around β-amyloid plaques, and surrounding vessels with β-amyloid angiopathy; they were also distributed randomly in the cerebral cortex and white matter. YKL-40 overexpression appeared as a pre-clinical event as demonstrated in experimental models of prion diseases and AD pathology. CSF YKL-40 levels were measured in a cohort of 288 individuals, including neurological controls (NC) and patients diagnosed with different types of dementia. Compared to NC, increased YKL-40 levels were detected in sCJD (p < 0.001, AUC = 0.92) and AD (p < 0.001, AUC = 0.77) but not in vascular dementia (VaD) (p > 0.05, AUC = 0.71) or in DLB/Parkinson’s disease dementia (PDD) (p > 0.05, AUC = 0.70). Further, two independent patient cohorts were used to validate the increased CSF YKL-40 levels in sCJD. Additionally, increased YKL-40 levels were found in genetic prion diseases associated with the PRNP-D178N (Fatal Familial Insomnia) and PRNP-E200K mutations. Conclusions Our results unequivocally demonstrate that in neurodegenerative dementias, YKL-40 is a disease-specific marker of neuroinflammation showing its highest levels in prion diseases. Therefore, YKL-40 quantification might have a potential for application in the evaluation of therapeutic intervention in dementias with a neuroinflammatory component."],["dc.description.sponsorship","Open-Access-Publikationsfonds 2017"],["dc.identifier.doi","10.1186/s13024-017-0226-4"],["dc.identifier.purl","https://resolver.sub.uni-goettingen.de/purl?gs-1/14995"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/59135"],["dc.language.iso","en"],["dc.notes.intern","Merged from goescholar"],["dc.notes.intern","In goescholar not merged with http://resolver.sub.uni-goettingen.de/purl?gs-1/15151 but duplicate"],["dc.rights","CC BY 4.0"],["dc.rights.access","openAccess"],["dc.rights.uri","https://creativecommons.org/licenses/by/4.0"],["dc.subject.ddc","610"],["dc.title","YKL-40 in the brain and cerebrospinal fluid of neurodegenerative dementias"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.version","published_version"],["dspace.entity.type","Publication"]]Details DOI2016Conference Abstract [["dc.bibliographiccitation.journal","Prion"],["dc.bibliographiccitation.volume","10"],["dc.contributor.author","Ferrer, Isidro"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Frau-Mendez, Lida"],["dc.contributor.author","Fernandez-Vega, Ivan"],["dc.contributor.author","Thune, Katrin"],["dc.contributor.author","Antonio del Rio, Jose"],["dc.contributor.author","Schmizt, Matthias"],["dc.contributor.author","Ansoleaga, Belen"],["dc.contributor.author","Gotzmann, Nadine"],["dc.contributor.author","Cramm, Maria"],["dc.contributor.author","Zerr, Inga"],["dc.contributor.author","Jose Zarranz, Juan"],["dc.date.accessioned","2018-11-07T10:20:26Z"],["dc.date.available","2018-11-07T10:20:26Z"],["dc.date.issued","2016"],["dc.format.extent","S83"],["dc.identifier.isi","000374656300119"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/41890"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Taylor & Francis Inc"],["dc.publisher.place","Philadelphia"],["dc.relation.issn","1933-690X"],["dc.relation.issn","1933-6896"],["dc.title","Identification of new molecular alterations in Fatal Familial Insomnia"],["dc.type","conference_abstract"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]Details WOS2016Journal Article [["dc.bibliographiccitation.firstpage","6412"],["dc.bibliographiccitation.issue","8"],["dc.bibliographiccitation.journal","Molecular Neurobiology"],["dc.bibliographiccitation.lastpage","6425"],["dc.bibliographiccitation.volume","54"],["dc.contributor.author","Mata, Agata"],["dc.contributor.author","Urrea, Laura"],["dc.contributor.author","Vilches, Silvia"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Thüne, Katrin"],["dc.contributor.author","Espinosa, Juan-Carlos"],["dc.contributor.author","Andréoletti, Olivier"],["dc.contributor.author","Sevillano, Alejandro M."],["dc.contributor.author","Torres, Juan MarÃa"],["dc.contributor.author","Requena, Jesús RodrÃguez"],["dc.contributor.author","Zerr, Inga"],["dc.contributor.author","Ferrer, Isidro"],["dc.contributor.author","GavÃn, Rosalina"],["dc.contributor.author","del RÃo, José Antonio"],["dc.date.accessioned","2020-12-10T14:14:24Z"],["dc.date.available","2020-12-10T14:14:24Z"],["dc.date.issued","2016"],["dc.identifier.doi","10.1007/s12035-016-0177-8"],["dc.identifier.eissn","1559-1182"],["dc.identifier.issn","0893-7648"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/71341"],["dc.language.iso","en"],["dc.notes.intern","DOI Import GROB-354"],["dc.title","Reelin Expression in Creutzfeldt-Jakob Disease and Experimental Models of Transmissible Spongiform Encephalopathies"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dspace.entity.type","Publication"]]Details DOI2020Journal Article Research Paper [["dc.bibliographiccitation.firstpage","8680"],["dc.bibliographiccitation.issue","22"],["dc.bibliographiccitation.journal","International Journal of Molecular Sciences"],["dc.bibliographiccitation.volume","21"],["dc.contributor.affiliation","Andrés-Benito, Pol; \t\t \r\n\t\t Department of Pathology and Experimental Therapeutics, University of Barcelona, Feixa Llarga s/n, 08907 L’Hospitalet de Llobregat, Barcelona, Spain, pol.andres.benito@gmail.com\t\t \r\n\t\t Biomedical Network Research Center on Neurodegenerative Diseases (CIBERNED), Institute Carlos III, Feixa Llarga s/n, 08907 L’Hospitalet de Llobregat, Barcelona, Spain, pol.andres.benito@gmail.com\t\t \r\n\t\t Bellvitge Biomedical Research Institute (IDIBELL), 08907 L’Hospitalet de Llobregat, Barcelona, Spain, pol.andres.benito@gmail.com\t\t \r\n\t\t Institute of Neurosciences, University of Barcelona, 08907 L’Hospitalet de Llobregat, Barcelona, Spain, pol.andres.benito@gmail.com\t\t \r\n\t\t International Initiative for Treatment and Research Initiative to Cure ALS (TRICALS), Bellvitge University Hospital, 08907 Hospitalet de Llobregat, Spain, pol.andres.benito@gmail.com"],["dc.contributor.affiliation","Povedano, Mònica; \t\t \r\n\t\t International Initiative for Treatment and Research Initiative to Cure ALS (TRICALS), Bellvitge University Hospital, 08907 Hospitalet de Llobregat, Spain, mpovedano@bellvitgehospital.cat\t\t \r\n\t\t Functional Unit of Amyotrophic Lateral Sclerosis (UFELA), Service of Neurology, Bellvitge University Hospital, 08907 L’Hospitalet de Llobregat, Barcelona, Spain, mpovedano@bellvitgehospital.cat"],["dc.contributor.affiliation","DomÃnguez, Raúl; \t\t \r\n\t\t International Initiative for Treatment and Research Initiative to Cure ALS (TRICALS), Bellvitge University Hospital, 08907 Hospitalet de Llobregat, Spain, rdominguez@bellvitgehospital.cat"],["dc.contributor.affiliation","Marco, Carla; \t\t \r\n\t\t International Initiative for Treatment and Research Initiative to Cure ALS (TRICALS), Bellvitge University Hospital, 08907 Hospitalet de Llobregat, Spain, carlamarcocazcarra@gmail.com"],["dc.contributor.affiliation","Colomina, Maria J.; \t\t \r\n\t\t Anesthesia and Critical Care Department, Bellvitge University Hospital-University of Barcelona, 08907 L’Hospitalet de Llobregat, Barcelona, Spain, Mjcolomina@gmail.com"],["dc.contributor.affiliation","López-Pérez, Óscar; \t\t \r\n\t\t Biomedical Network Research Center on Neurodegenerative Diseases (CIBERNED), Institute Carlos III, Feixa Llarga s/n, 08907 L’Hospitalet de Llobregat, Barcelona, Spain, oscarlzpz@gmail.com"],["dc.contributor.affiliation","Santana, Isabel; \t\t \r\n\t\t Neurology Department, CHUC—Centro Hospitalar e Universitário de Coimbra, CNC—Center for Neuroscience and Cell Biology; and Faculty of Medicine, University of Coimbra, 3000-456 Coimbra, Portugal, isabeljsantana@gmail.com"],["dc.contributor.affiliation","Baldeiras, Inês; \t\t \r\n\t\t Neurology Department, CHUC—Centro Hospitalar e Universitário de Coimbra, CNC—Center for Neuroscience and Cell Biology; and Faculty of Medicine, University of Coimbra, 3000-456 Coimbra, Portugal, ines.baldeiras@sapo.pt"],["dc.contributor.affiliation","MartÃnez-Yelámos, Sergio; \t\t \r\n\t\t Multiple Sclerosis Unit, Service of Neurology, Bellvitge University Hospital, 08907 L’Hospitalet de Llobregat, Barcelona, Spain, smartinezy@bellvitgehospital.cat"],["dc.contributor.affiliation","Zerr, Inga; \t\t \r\n\t\t Department of Neurology, University Medical Center Göttingen, 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","Llorens, Franc; \t\t \r\n\t\t Department of Pathology and Experimental Therapeutics, University of Barcelona, Feixa Llarga s/n, 08907 L’Hospitalet de Llobregat, Barcelona, Spain, franc.llorens@gmail.com\t\t \r\n\t\t Biomedical Network Research Center on Neurodegenerative Diseases (CIBERNED), Institute Carlos III, Feixa Llarga s/n, 08907 L’Hospitalet de Llobregat, Barcelona, Spain, franc.llorens@gmail.com\t\t \r\n\t\t Bellvitge Biomedical Research Institute (IDIBELL), 08907 L’Hospitalet de Llobregat, Barcelona, Spain, franc.llorens@gmail.com\t\t \r\n\t\t Institute of Neurosciences, University of Barcelona, 08907 L’Hospitalet de Llobregat, Barcelona, Spain, franc.llorens@gmail.com"],["dc.contributor.affiliation","Fernández-Irigoyen, JoaquÃn; \t\t \r\n\t\t IDISNA, Navarra Institute for Health Research, 31008 Pamplona, Spain, joaquin.fernandez.irigoyen@navarra.es\t\t \r\n\t\t Clinical Neuroproteomics Unit, Proteomics Platform, Proteored-ISCIII, Navarrabiomed, Complejo Hospitalario de Navarra (CHN), Universidad Pública de Navarra (UPNA), 31008 Pamplona, Spain, joaquin.fernandez.irigoyen@navarra.es"],["dc.contributor.affiliation","SantamarÃa, Enrique; \t\t \r\n\t\t IDISNA, Navarra Institute for Health Research, 31008 Pamplona, Spain, enrique.santamaria.martinez@navarra.es\t\t \r\n\t\t Clinical Neuroproteomics Unit, Proteomics Platform, Proteored-ISCIII, Navarrabiomed, Complejo Hospitalario de Navarra (CHN), Universidad Pública de Navarra (UPNA), 31008 Pamplona, Spain, enrique.santamaria.martinez@navarra.es"],["dc.contributor.affiliation","Ferrer, Isidro; \t\t \r\n\t\t Department of Pathology and Experimental Therapeutics, University of Barcelona, Feixa Llarga s/n, 08907 L’Hospitalet de Llobregat, Barcelona, Spain, 8082ifa@gmail.com\t\t \r\n\t\t Biomedical Network Research Center on Neurodegenerative Diseases (CIBERNED), Institute Carlos III, Feixa Llarga s/n, 08907 L’Hospitalet de Llobregat, Barcelona, Spain, 8082ifa@gmail.com\t\t \r\n\t\t Bellvitge Biomedical Research Institute (IDIBELL), 08907 L’Hospitalet de Llobregat, Barcelona, Spain, 8082ifa@gmail.com\t\t \r\n\t\t Institute of Neurosciences, University of Barcelona, 08907 L’Hospitalet de Llobregat, Barcelona, Spain, 8082ifa@gmail.com\t\t \r\n\t\t International Initiative for Treatment and Research Initiative to Cure ALS (TRICALS), Bellvitge University Hospital, 08907 Hospitalet de Llobregat, Spain, 8082ifa@gmail.com\t\t \r\n\t\t Neuropathology, Pathologic Anatomy Service, Bellvitge University Hospital, 08907 L’Hospitalet de Llobregat, Barcelona, Spain, 8082ifa@gmail.com"],["dc.contributor.author","Andrés-Benito, Pol"],["dc.contributor.author","Povedano, Mònica"],["dc.contributor.author","DomÃnguez, Raúl"],["dc.contributor.author","Marco, Carla"],["dc.contributor.author","Colomina, Maria J."],["dc.contributor.author","López-Pérez, Óscar"],["dc.contributor.author","Santana, Isabel"],["dc.contributor.author","Baldeiras, Inês"],["dc.contributor.author","MartÃnez-Yelámos, Sergio"],["dc.contributor.author","Zerr, Inga"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Fernández-Irigoyen, JoaquÃn"],["dc.contributor.author","SantamarÃa, Enrique"],["dc.contributor.author","Ferrer, Isidro"],["dc.date.accessioned","2021-04-14T08:31:05Z"],["dc.date.available","2021-04-14T08:31:05Z"],["dc.date.issued","2020"],["dc.date.updated","2022-09-05T14:19:42Z"],["dc.identifier.doi","10.3390/ijms21228680"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/83483"],["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","Increased C-X-C Motif Chemokine Ligand 12 Levels in Cerebrospinal Fluid as a Candidate Biomarker in Sporadic Amyotrophic Lateral Sclerosis"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.subtype","original_ja"],["dc.type.version","published_version"],["dspace.entity.type","Publication"]]Details DOI2017Journal Article [["dc.bibliographiccitation.artnumber","89"],["dc.bibliographiccitation.journal","Frontiers in neurology"],["dc.bibliographiccitation.volume","8"],["dc.contributor.author","Garcia-Esparcia, Paula"],["dc.contributor.author","López-González, Irene"],["dc.contributor.author","Grau-Rivera, Oriol"],["dc.contributor.author","GarcÃa-Garrido, MarÃa Francisca"],["dc.contributor.author","Konetti, Anusha"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Zafar, Saima"],["dc.contributor.author","Carmona, Margarita"],["dc.contributor.author","Del Rio, José Antonio"],["dc.contributor.author","Zerr, Inga"],["dc.contributor.author","Gelpi, Ellen"],["dc.contributor.author","Ferrer, Isidro"],["dc.date.accessioned","2019-07-09T11:44:52Z"],["dc.date.available","2019-07-09T11:44:52Z"],["dc.date.issued","2017"],["dc.description.abstract","OBJECTIVES: The goal of this study was to assess mitochondrial function, energy, and purine metabolism, protein synthesis machinery from the nucleolus to the ribosome, inflammation, and expression of newly identified ectopic olfactory receptors (ORs) and taste receptors (TASRs) in the frontal cortex of typical cases of dementia with Lewy bodies (DLB) and cases with rapid clinical course (rpDLB: 2 years or less) compared with middle-aged non-affected individuals, in order to learn about the biochemical abnormalities underlying Lewy body pathology. METHODS: Real-time quantitative PCR, mitochondrial enzymatic assays, and analysis of β-amyloid, tau, and synuclein species were used. RESULTS: The main alterations in DLB and rpDLB, which are more marked in the rapidly progressive forms, include (i) deregulated expression of several mRNAs and proteins of mitochondrial subunits, and reduced activity of complexes I, II, III, and IV of the mitochondrial respiratory chain; (ii) reduced expression of selected molecules involved in energy metabolism and increased expression of enzymes involved in purine metabolism; (iii) abnormal expression of nucleolar proteins, rRNA18S, genes encoding ribosomal proteins, and initiation factors of the transcription at the ribosome; (iv) discrete inflammation; and (v) marked deregulation of brain ORs and TASRs, respectively. Severe mitochondrial dysfunction involving activity of four complexes, minimal inflammatory responses, and dramatic altered expression of ORs and TASRs discriminate DLB from Alzheimer's disease. Altered solubility and aggregation of α-synuclein, increased β-amyloid bound to membranes, and absence of soluble tau oligomers are common in DLB and rpDLB. Low levels of soluble β-amyloid are found in DLB. However, increased soluble β-amyloid 1-40 and β-amyloid 1-42, and increased TNFα mRNA and protein expression, distinguish rpDLB. CONCLUSION: Molecular alterations in frontal cortex in DLB involve key biochemical pathways such as mitochondria and energy metabolism, protein synthesis, purine metabolism, among others and are accompanied by discrete innate inflammatory response."],["dc.identifier.doi","10.3389/fneur.2017.00089"],["dc.identifier.pmid","28348546"],["dc.identifier.purl","https://resolver.sub.uni-goettingen.de/purl?gs-1/14942"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/59117"],["dc.language.iso","en"],["dc.notes.intern","Merged from goescholar"],["dc.relation.issn","1664-2295"],["dc.rights","CC BY 4.0"],["dc.rights.uri","https://creativecommons.org/licenses/by/4.0"],["dc.subject.ddc","610"],["dc.title","Dementia with Lewy Bodies: Molecular Pathology in the Frontal Cortex in Typical and Rapidly Progressive Forms."],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.version","published_version"],["dspace.entity.type","Publication"]]Details DOI PMID PMC2016Journal Article [["dc.bibliographiccitation.firstpage","2417"],["dc.bibliographiccitation.issue","12"],["dc.bibliographiccitation.journal","Human Molecular Genetics"],["dc.bibliographiccitation.lastpage","2436"],["dc.bibliographiccitation.volume","25"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Thuene, Katrin"],["dc.contributor.author","Schmitz, Matthias"],["dc.contributor.author","Ansoleaga, Belen"],["dc.contributor.author","Frau-Mendez, Margalida A."],["dc.contributor.author","Cramm, Maria"],["dc.contributor.author","Tahir, Waqas"],["dc.contributor.author","Gotzmann, Nadine"],["dc.contributor.author","Berjaoui, Sara"],["dc.contributor.author","Carmona, Margarita"],["dc.contributor.author","Silva, Christopher J."],["dc.contributor.author","Fernandez-Vega, Ivan"],["dc.contributor.author","Jose Zarranz, Juan"],["dc.contributor.author","Zerr, Inga"],["dc.contributor.author","Ferrer, Isidro"],["dc.date.accessioned","2018-11-07T10:12:45Z"],["dc.date.available","2018-11-07T10:12:45Z"],["dc.date.issued","2016"],["dc.description.abstract","Fatal familial insomnia is a rare disease caused by a D178N mutation in combination with methionine (Met) at codon 129 in the mutated allele of PRNP (D178N-129M haplotype). FFI is manifested by sleep disturbances with insomnia, autonomic disorders and spontaneous and evoked myoclonus, among other symptoms. This study describes new neuropathological and biochemical observations in a series of eight patients with FFI. The mediodorsal and anterior nuclei of the thalamus have severe neuronal loss and marked astrocytic gliosis in every case, whereas the entorhinal cortex is variably affected. Spongiform degeneration only occurs in the entorhinal cortex. Synaptic and fine granular proteinase K digestion (PrPres) immunoreactivity is found in the entorhinal cortex but not in the thalamus. Interleukin 6, interleukin 10 receptor alpha subunit, colony stimulating factor 3 receptor and toll-like receptor 7 mRNA expression increases in the thalamus in FFI. PrPc levels are significantly decreased in the thalamus, entorhinal cortex and cerebellum in FFI. This is accompanied by a particular PrPc and PrPres band profile. Altered PrP solubility consistent with significantly reduced PrP levels in the cytoplasmic fraction and increased PrP levels in the insoluble fraction are identified in FFI cases. Amyloid-like deposits are only seen in the entorhinal cortex. The RT-QuIC assay reveals that all the FFI samples of the entorhinal cortex are positive, whereas the thalamus is positive only in three cases and the cerebellumin two cases. The present findings unveil particular neuropathological and neuroinflammatory profiles in FFI and novel characteristics of natural prion protein in FFI, altered PrPres and Scrapie PrP (abnormal and pathogenic PrP) patterns and region-dependent putative capacity of PrP seeding."],["dc.identifier.doi","10.1093/hmg/ddw108"],["dc.identifier.isi","000393062900005"],["dc.identifier.pmid","27056979"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/40298"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Oxford Univ Press"],["dc.relation.issn","1460-2083"],["dc.relation.issn","0964-6906"],["dc.title","Identification of new molecular alterations in fatal familial insomnia"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]Details DOI PMID PMC WOS2020Journal Article [["dc.bibliographiccitation.issue","1"],["dc.bibliographiccitation.journal","Nature Communications"],["dc.bibliographiccitation.volume","11"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Hermann, Peter"],["dc.contributor.author","Villar-Piqué, Anna"],["dc.contributor.author","Diaz-Lucena, Daniela"],["dc.contributor.author","Nägga, Katarina"],["dc.contributor.author","Hansson, Oskar"],["dc.contributor.author","Santana, Isabel"],["dc.contributor.author","Schmitz, Matthias"],["dc.contributor.author","Schmidt, Christian"],["dc.contributor.author","Varges, Daniela"],["dc.contributor.author","Goebel, Stefan"],["dc.contributor.author","Dumurgier, Julien"],["dc.contributor.author","Zetterberg, Henrik"],["dc.contributor.author","Blennow, Kaj"],["dc.contributor.author","Paquet, Claire"],["dc.contributor.author","Baldeiras, Inês"],["dc.contributor.author","Ferrer, Isidro"],["dc.contributor.author","Zerr, Inga"],["dc.date.accessioned","2021-04-14T08:27:37Z"],["dc.date.available","2021-04-14T08:27:37Z"],["dc.date.issued","2020"],["dc.identifier.doi","10.1038/s41467-020-14373-2"],["dc.identifier.purl","https://resolver.sub.uni-goettingen.de/purl?gs-1/17201"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/82349"],["dc.language.iso","en"],["dc.notes.intern","DOI Import GROB-399"],["dc.notes.intern","Merged from goescholar"],["dc.relation.eissn","2041-1723"],["dc.rights","CC BY 4.0"],["dc.rights.uri","https://creativecommons.org/licenses/by/4.0"],["dc.title","Cerebrospinal fluid lipocalin 2 as a novel biomarker for the differential diagnosis of vascular dementia"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.version","published_version"],["dspace.entity.type","Publication"]]Details DOI2016Review [["dc.bibliographiccitation.firstpage","36"],["dc.bibliographiccitation.journal","Progress in Neurobiology"],["dc.bibliographiccitation.lastpage","53"],["dc.bibliographiccitation.volume","138"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Schmitz, Matthias"],["dc.contributor.author","Ferrer, Isidro"],["dc.contributor.author","Zerr, Inga"],["dc.date.accessioned","2018-11-07T10:17:05Z"],["dc.date.available","2018-11-07T10:17:05Z"],["dc.date.issued","2016"],["dc.description.abstract","Neurodegenerative diseases with abnormal protein aggregates such as Alzheimer's disease, tauopathies, synucleinopathies, and prionopathies, together with vascular encephalopathies, are cause of cognitive impairment and dementia, Identification of reliable biomarkers in biological fluids, particularly in the cerebrospinal fluid (CSF), is of extreme importance in optimizing the precise early clinical diagnosis of distinct entities and predicting the outcome in particular settings. In addition, the study of CSF biomarkers is useful to identify and monitor the underlying pathological processes developing in the central nervous system of affected individuals. Evidence suggests that levels of key CSF molecules correlate, in some circumstances, with prediction, disease progression, and severity of cognitive decline. Correlation of CSF markers and underlying pathological molecular substrates in brain is an exciting field for further study. However, while some dementias such as Creutzfeldt-Jakob disease have accurate CSF biomarkers, other disease types such as dementia with Lewy bodies, vascular dementia, and frontotemporal dementia lack reliable biomarkers for their specific clinical diagnosis. (C) 2016 Elsevier Ltd. All rights reserved."],["dc.identifier.doi","10.1016/j.pneurobio.2016.03.003"],["dc.identifier.isi","000374919700003"],["dc.identifier.pmid","27016008"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/41164"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Pergamon-elsevier Science Ltd"],["dc.relation.issn","0301-0082"],["dc.title","CSF biomarkers in neurodegenerative and vascular dementias"],["dc.type","review"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]Details DOI PMID PMC WOS