Full Text Journal Articles by
Author Franc Llorens

Advertisement

Find full text journal articles






Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease.

Peter Hermann, Brian Appleby, Jean-Philippe Brandel, Byron Caughey, Steven Collins, Michael D Geschwind, Alison Green, Stephane Haïk, Gabor G Kovacs, Anna Ladogana, Franc Llorens, Simon Mead, Noriyuki Nishida, Suvankar Pal, Piero Parchi, Maurizio Pocchiari, Katsuya Satoh, Gianluigi Zanusso, Inga Zerr,

Sporadic Creutzfeldt-Jakob disease is a fatal neurodegenerative disease caused by misfolded prion proteins (PrP<sup>Sc</sup>). Effective therapeutics are currently not available and accurate diagnosis can be challenging. Clinical diagnostic criteria use a combination of characteristic neuropsychiatric symptoms, CSF proteins 14-3-3, MRI, and EEG. Supportive biomarkers, such as high CSF total tau, ... Read more >>

Lancet Neurol (The Lancet. Neurology)
[2021, 20(3):235-246]

Cited: 0 times

View full text PDF listing >>



Ecto-GPR37: a potential biomarker for Parkinson's disease.

Xavier Morató, Paula Garcia-Esparcia, Josep Argerich, Franc Llorens, Inga Zerr, Wojciech Paslawski, Eva Borràs, Eduard Sabidó, Ulla E Petäjä-Repo, Víctor Fernández-Dueñas, Isidro Ferrer, Per Svenningsson, Francisco Ciruela,

<h4>Objective</h4>α-Synuclein has been studied as a potential biomarker for Parkinson's disease (PD) with no concluding results. Accordingly, there is an urgent need to find out reliable specific biomarkers for PD. GPR37 is an orphan G protein-coupled receptor that toxically accumulates in autosomal recessive juvenile parkinsonism. Here, we investigated whether GPR37 ... Read more >>

Transl Neurodegener (Translational neurodegeneration)
[2021, 10(1):8]

Cited: 0 times

View full text PDF listing >>



Advertisement

Huntington's disease brain-derived small RNAs recapitulate associated neuropathology in mice.

Jordi Creus-Muncunill, Anna Guisado-Corcoll, Veronica Venturi, Lorena Pantano, Georgia Escaramís, Marta García de Herreros, Maria Solaguren-Beascoa, Ana Gámez-Valero, Cristina Navarrete, Mercè Masana, Franc Llorens, Daniela Diaz-Lucena, Esther Pérez-Navarro, Eulàlia Martí,

Progressive motor alterations and selective death of striatal medium spiny neurons (MSNs) are key pathological hallmarks of Huntington's disease (HD), a neurodegenerative condition caused by a CAG trinucleotide repeat expansion in the coding region of the huntingtin (HTT) gene. Most research has focused on the pathogenic effects of the resultant ... Read more >>

Acta Neuropathol (Acta neuropathologica)
[2021, :]

Cited: 0 times

View full text PDF listing >>



Optimization of the Real-Time Quaking-Induced Conversion Assay for Prion Disease Diagnosis.

Inga Zerr, Maria Cramm, Susana Margarida da Silva Correia, Saima Zafar, Anna Villar-Piqué, Franc Llorens, Matthias Schmitz,

The real-time quaking-induced conversion (RT-QuIC) assay is a highly reproducible and robust methodology exhibiting an excellent pre-mortem diagnostic accuracy for prion diseases. However, the protocols might be time-consuming and improvement of the detection technology is needed. In the present study, we investigated the influence of a pre-analytical cerebrospinal fluid (CSF) ... Read more >>

Front Bioeng Biotechnol (Frontiers in bioengineering and biotechnology)
[2020, 8:586890]

Cited: 0 times

View full text PDF listing >>



Ecto-GPR37: a potential biomarker for Parkinson’s disease

Xavier Morató, Paula Garcia-Esparcia, Josep Argerich, Franc Llorens, Inga Zerr, Wojciech Paslawski, Eva Borràs, Eduard Sabidó, Ulla E. Petäjä-Repo, Víctor Fernández-Dueñas, Isidro Ferrer, Per Svenningsson, Francisco Ciruela,

<h4>Objective</h4> α-Synuclein has been studied as a potential biomarker for Parkinson’s disease (PD) with no concluding results. Accordingly, there is an urgent need to find out reliable specific biomarkers for PD. GPR37 is an orphan G protein-coupled receptor that toxically accumulates in autosomal recessive juvenile parkinsonism. Here, we investigated whether ... Read more >>

Transl Neurodegener (Translational neurodegeneration)
[2021, 10(1):]

Cited: 0 times

View full text PDF listing >>



Cerebrospinal Fluid Mitochondrial DNA in Rapid and Slow Progressive Forms of Alzheimer's Disease.

Petar Podlesniy, Franc Llorens, Margalida Puigròs, Nuria Serra, Diego Sepúlveda-Falla, Christian Schmidt, Peter Hermann, Inga Zerr, Ramon Trullas,

Alzheimer's type dementia (AD) exhibits clinical heterogeneity, as well as differences in disease progression, as a subset of patients with a clinical diagnosis of AD progresses more rapidly (rpAD) than the typical AD of slow progression (spAD). Previous findings indicate that low cerebrospinal fluid (CSF) content of cell-free mitochondrial DNA ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2020, 21(17):]

Cited: 0 times

View full text PDF listing >>



A prognostic model for overall survival in sporadic Creutzfeldt-Jakob disease.

Franc Llorens, Nicole Rübsamen, Peter Hermann, Matthias Schmitz, Anna Villar-Piqué, Stefan Goebel, André Karch, Inga Zerr,

<b>Introduction: </b>We developed a prognostic model for overall survival after diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) using data from a German surveillance study.<br><br><b>Methods: </b>We included 1226 sCJD cases (median age 66 years, range 19-89 years; 56.8% women with information on age, sex, codon 129 genotype, 14-3-3 in the cerebrospinal fluid ... Read more >>

Alzheimers Dement (Alzheimer's & dementia : the journal of the Alzheimer's Association)
[2020, 16(10):1438-1447]

Cited: 0 times

View full text PDF listing >>



Disease-Specific Changes in Reelin Protein and mRNA in Neurodegenerative Diseases.

Laia Lidón, Laura Urrea, Franc Llorens, Vanessa Gil, Ignacio Alvarez, Monica Diez-Fairen, Miguel Aguilar, Pau Pastor, Inga Zerr, Daniel Alcolea, Alberto Lleó, Enric Vidal, Rosalina Gavín, Isidre Ferrer, Jose Antonio Del Rio,

Reelin is an extracellular glycoprotein that modulates neuronal function and synaptic plasticity in the adult brain. Decreased levels of Reelin activity have been postulated as a key factor during neurodegeneration in Alzheimer´s disease (AD) and in aging. Thus, changes in levels of full-length Reelin and Reelin fragments have been revealed ... Read more >>

Cells (Cells)
[2020, 9(5):]

Cited: 0 times

View full text PDF listing >>



A new tetra-plex fluorimetric assay for the quantification of cerebrospinal fluid β-amyloid42, total-tau, phospho-tau and α-synuclein in the differential diagnosis of neurodegenerative dementia.

Daniela Diaz-Lucena, Geòrgia Escaramis, Anna Villar-Piqué, Peter Hermann, Matthias Schmitz, Daniela Varges, Isabel Santana, José Antonio Del Rio, Eulàlia Martí, Isidre Ferrer, Inês Baldeiras, Inga Zerr, Franc Llorens,

BACKGROUND:Differential diagnosis of neurodegenerative dementia is currently supported by biomarkers including cerebrospinal fluid (CSF) tests. Among them, CSF total-tau (t-tau), phosphorylated tau (p-tau) and β-amyloid42 (Aβ42) are considered core biomarkers of neurodegeneration. In the present work, we hypothesize that simultaneous assessment of these biomarkers together with CSF α-synuclein (α-syn) will ... Read more >>

J Neurol (Journal of neurology)
[2020, 267(9):2567-2581]

Cited: 0 times

View full text PDF listing >>



Cerebrospinal fluid neurogranin as a new player in prion disease diagnosis and prognosis.

Anna Villar-Pique, Inga Zerr, Franc Llorens,

Neural Regen Res (Neural regeneration research)
[2020, 15(5):861-862]

Cited: 0 times

View full text PDF listing >>



BAMBI and CHGA in Prion Diseases: Neuropathological Assessment and Potential Role as Disease Biomarkers.

Óscar López-Pérez, Marcos Bernal-Martín, Adelaida Hernaiz, Franc Llorens, Marina Betancor, Alicia Otero, Janne Markus Toivonen, Pilar Zaragoza, Inga Zerr, Juan José Badiola, Rosa Bolea, Inmaculada Martín-Burriel,

Prion diseases affect both animals and humans. Research in the natural animal model of the disease could help in the understanding of neuropathological mechanisms and in the development of biomarkers for human pathologies. For this purpose, we studied the expression of 10 genes involved in prion propagation in vitro in ... Read more >>

Biomolecules (Biomolecules)
[2020, 10(5):]

Cited: 1 time

View full text PDF listing >>



Diagnostic Accuracy of Prion Disease Biomarkers in Iatrogenic Creutzfeldt-Jakob Disease.

Franc Llorens, Anna Villar-Piqué, Peter Hermann, Matthias Schmitz, Olga Calero, Christiane Stehmann, Shannon Sarros, Fabio Moda, Isidre Ferrer, Anna Poleggi, Maurizio Pocchiari, Marcella Catania, Sigrid Klotz, Carl O'Regan, Francesca Brett, Josephine Heffernan, Anna Ladogana, Steven J Collins, Miguel Calero, Gabor G Kovacs, Inga Zerr,

Human prion diseases are classified into sporadic, genetic, and acquired forms. Within this last group, iatrogenic Creutzfeldt-Jakob disease (iCJD) is caused by human-to-human transmission through surgical and medical procedures. After reaching an incidence peak in the 1990s, it is believed that the iCJD historical period is probably coming to an ... Read more >>

Biomolecules (Biomolecules)
[2020, 10(2):]

Cited: 0 times

View full text PDF listing >>



A new tetra-plex fluorimetric assay for the quantification of cerebrospinal fluid β-amyloid42, total-tau, phospho-tau and α-synuclein in the differential diagnosis of neurodegenerative dementia.

Daniela Diaz Lucena, Geòrgia Escaramis, Anna Villar-Piqué, Peter Hermann, Matthias Schmitz, Daniela Varges, Isabel Santana, José Antonio del Rio, Eulàlia Martí, Isidre Ferrer, Inês Baldeiras, Inga Zerr, Franc Llorens,

<h4>Background: </h4> Differential diagnosis of neurodegenerative dementia is currently supported by biomarkers including cerebrospinal fluid (CSF) tests. Among them, CSF total-tau (t-tau), phosphorylated tau (p-tau) and β-amyloid42 (Aβ42) are considered core biomarkers of neurodegeneration. In the present work, we hypothesize that simultaneous assessment of these biomarkers together with CSF α-synuclein ... Read more >>

()
[, :]

Cited: 0 times

View full text PDF listing >>



Effect of the micro-environment on α-synuclein conversion and implication in seeded conversion assays.

Niccolo Candelise, Matthias Schmitz, Katrin Thüne, Maria Cramm, Alberto Rabano, Saima Zafar, Erik Stoops, Hugo Vanderstichele, Anna Villar-Pique, Franc Llorens, Inga Zerr,

Background:α-Synuclein is a small soluble protein, whose physiological function in the healthy brain is poorly understood. Intracellular inclusions of α-synuclein, referred to as Lewy bodies (LBs), are pathological hallmarks of α-synucleinopathies, such as Parkinson's disease (PD) or dementia with Lewy bodies (DLB). Main body:Understanding of the molecular basis as well ... Read more >>

Transl Neurodegener (Translational neurodegeneration)
[2020, 9:5]

Cited: 1 time

View full text PDF listing >>



Cerebrospinal fluid non-phosphorylated tau in the differential diagnosis of Creutzfeldt-Jakob disease: a comparative prospective study with 14-3-3.

Franc Llorens, Anna Villar-Piqué, Peter Hermann, Matthias Schmitz, Stefan Goebel, Katharina Waniek, Ingolf Lachmann, Inga Zerr,

Cerebrospinal fluid (CSF) non-phosphorylated tau (non-p-tau) is increased in sporadic Creutzfeldt-Jakob disease (CJD), but its accuracy in the differential diagnosis has not been previously established. Here, we first used a retrospective cohort of non-CJD (n = 135) and CJD (n = 137) cases to determine the optimal cutoff point for the discrimination of CJD ... Read more >>

J Neurol (Journal of neurology)
[2020, 267(2):543-550]

Cited: 0 times

View full text PDF listing >>



Validation of Poly(Propylene Imine) Glycodendrimers Towards Their Anti-prion Conversion Efficiency.

Matthias Schmitz, Niccolo Candelise, Eirini Kanata, Franc Llorens, Katrin Thüne, Anna Villar-Piqué, Susana Margarida da Silva Correia, Dimitra Dafou, Theodoros Sklaviadis, Dietmar Appelhans, Inga Zerr,

Prion diseases, such as the sporadic Creutzfeldt-Jakob disease (sCJD), are a class of fatal neurodegenerative disorders. Currently, there is no efficient treatment or therapy available. Hence, the search for molecules that may inhibit the conversion of the cellular prion protein (PrP<sup>C</sup>) into its pathological counterpart PrPScrapie (PrP<sup>Sc</sup>) is of great ... Read more >>

Mol Neurobiol (Molecular neurobiology)
[2020, 57(4):1863-1874]

Cited: 0 times

View full text PDF listing >>



Evaluation of Human Cerebrospinal Fluid Malate Dehydrogenase 1 as a Marker in Genetic Prion Disease Patients.

Inga Zerr, Anna Villar-Piqué, Vanda Edit Schmitz, Anna Poleggi, Maurizio Pocchiari, Raquel Sánchez-Valle, Miguel Calero, Olga Calero, Inês Baldeiras, Isabel Santana, Gabor G Kovacs, Franc Llorens, Matthias Schmitz,

The exploration of accurate diagnostic markers for differential diagnosis of neurodegenerative diseases is an ongoing topic. A previous study on cerebrospinal fluid (CSF)-mitochondrial malate dehydrogenase 1 (MDH1) in sporadic Creutzfeldt-Jakob disease (sCJD) patients revealed a highly significant upregulation of MDH1. Here, we measured the CSF levels of MDH1 via enzyme-linked ... Read more >>

Biomolecules (Biomolecules)
[2019, 9(12):]

Cited: 0 times

View full text PDF listing >>



The cellular prion protein and its derived fragments in human prion diseases and their role as potential biomarkers.

Katrin Thüne, Matthias Schmitz, Anna Villar-Piqué, Hermann Clemens Altmeppen, Markus Schlomm, Saima Zafar, Markus Glatzel, Franc Llorens, Inga Zerr,

<b>Introduction</b>: Human prion diseases are a heterogeneous group of incurable and debilitating conditions characterized by a progressive degeneration of the central nervous system. The conformational changes of the cellular prion protein and its formation into an abnormal isoform, spongiform degeneration, neuronal loss, and neuroinflammation are central to prion disease pathogenesis. ... Read more >>

Expert Rev Mol Diagn (Expert review of molecular diagnostics)
[2019, 19(11):1007-1018]

Cited: 1 time

View full text PDF listing >>



RNA editing alterations define manifestation of prion diseases.

Eirini Kanata, Franc Llorens, Dimitra Dafou, Athanasios Dimitriadis, Katrin Thüne, Konstantinos Xanthopoulos, Nikolaos Bekas, Juan Carlos Espinosa, Matthias Schmitz, Alba Marín-Moreno, Vincenzo Capece, Orr Shormoni, Olivier Andréoletti, Stefan Bonn, Juan María Torres, Isidre Ferrer, Inga Zerr, Theodoros Sklaviadis,

Prion diseases are fatal neurodegenerative disorders caused by misfolding of the normal prion protein into an infectious cellular pathogen. Clinically characterized by rapidly progressive dementia and accounting for 85% of human prion disease cases, sporadic Creutzfeldt-Jakob disease (sCJD) is the prevalent human prion disease. Although sCJD neuropathological hallmarks are well-known, ... Read more >>

Proc Natl Acad Sci U S A (Proceedings of the National Academy of Sciences of the United States of America)
[2019, 116(39):19727-19735]

Cited: 1 time

View full text PDF listing >>



Plasma YKL-40 in the spectrum of neurodegenerative dementia.

Anna Villar-Piqué, Matthias Schmitz, Peter Hermann, Stefan Goebel, Timothy Bunck, Daniela Varges, Isidre Ferrer, Joachim Riggert, Franc Llorens, Inga Zerr,

<h4>Background</h4>Increased plasma YKL-40 has been reported in Alzheimer's disease (AD), but its levels in other neurodegenerative diseases are unknown. Here, we aimed to investigate plasma YKL-40 in the spectrum of neurodegenerative dementias.<h4>Methods</h4>YKL-40 was quantified in the plasma of 315 cases, including healthy controls (HC), neurological disease controls (ND), AD, vascular ... Read more >>

J Neuroinflammation (Journal of neuroinflammation)
[2019, 16(1):145]

Cited: 1 time

View full text PDF listing >>



Domain-specific quantification of prion protein in cerebrospinal fluid by targeted mass spectrometry

Eric Vallabh Minikel, Eric Kuhn, Alexandra Cocco, Sonia Vallabh, Christina Hartigan, Andrew Reidenbach, Jiri Safar, Gregory Raymond, Michael McCarthy, Rhonda O’Keefe, Franc Llorens, Inga Zerr, Sabina Capellari, Piero Parchi, Stuart Schreiber, Steven Carr,

Therapies currently in preclinical development for prion disease seek to lower prion protein (PrP) expression in the brain. Trials of such therapies are likely to rely on quantification of PrP in cerebrospinal fluid (CSF) as a pharmacodynamic biomarker and possibly as a trial endpoint. Studies using PrP ELISA kits have ... Read more >>

()
[, :]

Cited: 1 time

View full text PDF listing >>



CSF neurogranin as a neuronal damage marker in CJD: a comparative study with AD.

Kaj Blennow, Daniela Diaz-Lucena, Henrik Zetterberg, Anna Villar-Pique, Andre Karch, Enric Vidal, Peter Hermann, Matthias Schmitz, Isidro Ferrer Abizanda, Inga Zerr, Franc Llorens,

<h4>Objective</h4>To investigate whether cerebrospinal fluid (CSF) neurogranin concentrations are altered in sporadic Creutzfeldt-Jakob disease (CJD), comparatively with Alzheimer's disease (AD), and associated with neuronal degeneration in brain tissue.<h4>Methods</h4>CSF neurogranin, total tau, neurofilament light (NFL) and 14-3-3 protein were measured in neurological controls (NCs, n=64), AD (n=46) and CJD (n=81). The ... Read more >>

J Neurol Neurosurg Psychiatry (Journal of neurology, neurosurgery, and psychiatry)
[2019, 90(8):846-853]

Cited: 6 times

View full text PDF listing >>



Seeding variability of different alpha synuclein strains in synucleinopathies.

Niccolò Candelise, Matthias Schmitz, Franc Llorens, Anna Villar-Piqué, Maria Cramm, Tobias Thom, Susana Margarida da Silva Correia, José Eriton Gomes da Cunha, Wiebke Möbius, Tiago F Outeiro, Valentina González Álvarez, Martina Banchelli, Cristiano D'Andrea, Marella de Angelis, Saima Zafar, Alberto Rabano, Paolo Matteini, Inga Zerr,

<h4>Objectives</h4>Currently, the exact reasons why different α-synucleinopathies exhibit variable pathologies and phenotypes are still unknown. A potential explanation may be the existence of distinctive α-synuclein conformers or strains. Here, we intend to analyze the seeding activity of dementia with Lewy bodies (DLB) and Parkinson's disease (PD) brain-derived α-synuclein seeds by ... Read more >>

Ann Neurol (Annals of neurology)
[2019, 85(5):691-703]

Cited: 18 times

View full text PDF listing >>



Prion protein quantification in human cerebrospinal fluid as a tool for prion disease drug development.

Sonia M Vallabh, Chloe K Nobuhara, Franc Llorens, Inga Zerr, Piero Parchi, Sabina Capellari, Eric Kuhn, Jacob Klickstein, Jiri G Safar, Flavia C Nery, Kathryn J Swoboda, Michael D Geschwind, Henrik Zetterberg, Steven E Arnold, Eric Vallabh Minikel, Stuart L Schreiber,

Reduction of native prion protein (PrP) levels in the brain is an attractive strategy for the treatment or prevention of human prion disease. Clinical development of any PrP-reducing therapeutic will require an appropriate pharmacodynamic biomarker: a practical and robust method for quantifying PrP, and reliably demonstrating its reduction in the ... Read more >>

Proc Natl Acad Sci U S A (Proceedings of the National Academy of Sciences of the United States of America)
[2019, 116(16):7793-7798]

Cited: 8 times

View full text PDF listing >>



Interlaboratory validation of cerebrospinal fluid α-synuclein quantification in the diagnosis of sporadic Creutzfeldt-Jakob disease.

Niels Kruse, Amanda Heslegrave, Vandana Gupta, Martha Foiani, Anna Villar-Piqué, Matthias Schmitz, Sylvain Lehmann, Charlotte Teunissen, Kaj Blennow, Henrik Zetterberg, Brit Mollenhauer, Inga Zerr, Franc Llorens,

<h4>Introduction</h4>Cerebrospinal fluid α-synuclein level is increased in sporadic Creutzfeldt-Jakob disease cases. However, the clinical value of this biomarker remains to be established. In this study, we have addressed the clinical validation parameters and the interlaboratory reproducibility by using an electrochemiluminescent assay.<h4>Methods</h4>Cerebrospinal fluid α-synuclein was quantified in a total of 188 ... Read more >>

Alzheimers Dement (Amst) (Alzheimer's & dementia (Amsterdam, Netherlands))
[2018, 10:461-470]

Cited: 2 times

View full text PDF listing >>



Advertisement

Disclaimer
1.2304 s