Llorens, Franc

[["dc.bibliographiccitation.firstpage","383"],["dc.bibliographiccitation.issue","5"],["dc.bibliographiccitation.journal","Prion"],["dc.bibliographiccitation.lastpage","393"],["dc.bibliographiccitation.volume","7"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Ansoleaga, Belen"],["dc.contributor.author","Garcia-Esparcia, Paula"],["dc.contributor.author","Zafar, Saima"],["dc.contributor.author","Grau-Rivera, Oriol"],["dc.contributor.author","Lopez-Gonzalez, Irene"],["dc.contributor.author","Blanco, Rosi"],["dc.contributor.author","Carmona, Margarita"],["dc.contributor.author","Yaguee, Jordi"],["dc.contributor.author","Nos, Carlos"],["dc.contributor.author","Antonio del Rio, Jose"],["dc.contributor.author","Gelpi, Ellen"],["dc.contributor.author","Zerr, Inga"],["dc.contributor.author","Ferrer, Isidre"],["dc.date.accessioned","2018-11-07T09:20:16Z"],["dc.date.available","2018-11-07T09:20:16Z"],["dc.date.issued","2013"],["dc.description.abstract","Creutzfeldt-Jakob disease (CJD) is a heterogenic neurodegenerative disorder associated with abnormal post-translational processing of cellular prion protein (PrPc). CJD displays distinctive clinical and pathological features which correlate with the genotype at the codon 129 (methionine or valine: M or V respectively) in the prion protein gene and with size of the protease-resistant core of the abnormal prion protein PrPsc (type 1: 20/21 kDa and type 2: 19 kDa). MM1 and VV2 are the most common sporadic CJD (sCJD) subtypes. PrP mRNA expression levels in the frontal cortex and cerebellum are reduced in sCJD in a form subtype-dependent. Total PrP protein levels and PrPsc levels in the frontal cortex and cerebellum accumulate differentially in sCJD MM1 and sCJD VV2 with no relation between PrPsc deposition and spongiform degeneration and neuron loss, but with microgliosis, and IL6 and TNF- response. In the CSF, reduced PrPc, the only form present in this compartment, occurs in sCJD MM1 and VV2. PrP mRNA expression is also reduced in the frontal cortex in advanced stages of Alzheimer disease, Lewy body disease, progressive supranuclear palsy, and frontotemporal lobe degeneration, but PrPc levels in brain varies from one disease to another. Reduced PrPc levels in CSF correlate with PrP mRNA expression in brain, which in turn reflects severity of degeneration in sCJD."],["dc.identifier.doi","10.4161/pri.26416"],["dc.identifier.isi","000327383600005"],["dc.identifier.pmid","24047819"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/28844"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Landes Bioscience"],["dc.relation.issn","1933-690X"],["dc.relation.issn","1933-6896"],["dc.title","PrP mRNA and protein expression in brain and PrPc in CSF in Creutzfeldt-Jakob disease MM1 and VV2"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","755"],["dc.bibliographiccitation.issue","8"],["dc.bibliographiccitation.journal","Journal of Neuropathology & Experimental Neurology"],["dc.bibliographiccitation.lastpage","769"],["dc.bibliographiccitation.volume","75"],["dc.contributor.author","Ansoleaga, Belen"],["dc.contributor.author","Garcia-Esparcia, Paula"],["dc.contributor.author","Llorens, Franc"],["dc.contributor.author","Hernandez-Ortega, Karina"],["dc.contributor.author","Carmona, Margarita"],["dc.contributor.author","Antonio del Rio, Jose"],["dc.contributor.author","Zerr, Inga"],["dc.contributor.author","Ferrer, Isidro"],["dc.date.accessioned","2018-11-07T10:10:51Z"],["dc.date.available","2018-11-07T10:10:51Z"],["dc.date.issued","2016"],["dc.description.abstract","Neuron loss, synaptic decline, and spongiform change are the hallmarks of sporadic Creutzfeldt-Jakob disease (sCJD), and may be related to deficiencies in mitochondria, energy metabolism, and protein synthesis. To investigate these relationships, we determined the expression levels of genes encoding subunits of the 5 protein complexes of the electron transport chain, proteins involved in energy metabolism, nucleolar and ribosomal proteins, and enzymes of purine metabolism in frontal cortex samples from 15 cases of sCJD MM1 and age-matched controls. We also assessed the protein expression levels of subunits of the respiratory chain, initiation and elongation translation factors of protein synthesis, and localization of selected mitochondrial components. We identified marked, generalized alterations of mRNA and protein expression of most subunits of all 5 mitochondrial respiratory chain complexes in sCJD cases. Expression of molecules involved in protein synthesis and purine metabolism were also altered in sCJD. These findings point to altered mRNA and protein expression of components of mitochondria, protein synthesis machinery, and purine metabolism as components of the pathogenesis of CJD."],["dc.identifier.doi","10.1093/jnen/nlw048"],["dc.identifier.isi","000383260100005"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/39938"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Lippincott Williams & Wilkins"],["dc.relation.issn","0022-3069"],["dc.title","Altered Mitochondria, Protein Synthesis Machinery, and Purine Metabolism Are Molecular Contributors to the Pathogenesis of Creutzfeldt-Jakob Disease"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]