Action Members are any researcher who participates actively in PROTEOSTASIS. All Members belong to one or more Working Group. Members can include researchers from COST Countries, Near Neighbour and International Partner Countries.

Michael John Clague

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University of Liverpool
http://pcwww.liv.ac.uk
United Kingdom
WG3
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Endocytosis, RTK trafficking and signaling. Deubiquitylation in pathways germane to cancer
Michael John Holdsworth

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University of Nottingham
http://sites.google.com
UK
WG1
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The N-end rule pathway of ubiquitin-mediated targeted proteolysis, oxygen and nitric oxide (NO) sensing, plant-environment interactions, abiotic and biotic stress responses, biochemical genetics.
Michal Sharon

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No website
Israel
WG1
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Speciality:My lab focuses on unravelling the regulatory mechanisms that coordinate the function of large protein complexes, by means of novel native mass spectrometry methods coupled with biochemistry and cell biology. Specifically, we focus on discovering the mechanisms that control the activity of molecular machines involved in the protein degradation pathway, aiming at: i) Understanding the regulatory principles that coordinate the 20S proteasome ubiquitin independent degradation pathway. ii) Investigating the underlying mechanisms that control the function of the COP9 signalosome (CSN), a complex involved in regulating the ubiquitination process. Skills: My lab develops and applies native mass spectrometry (MS) approaches aimed at understanding structure-function relationship of large protein complexes. Native MS has the ability to transfer intact protein complexes to the gas phase while maintaining weak interactions between subunits and associated biomolecules such as protein partners. This method enables deeper insights into the structural and functional properties of protein complexes, and is a central tool in studying the biology of large machines/complexes, providing details of subunit stoichiometry, composition, protein interaction partners, structural arrangement and overall architecture. The high sensitivity and, consequently, low sample requirement is a major advantage along with the speed of analysis which allows monitoring reactions in a time-resolved manner.
Mickael M. Cohen

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Institut de Biologie Physico-Chimique-CNRS. IBPC-CNRS
http://www.ibpc.fr
France
WG3
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mitochondrial dynamics. Current achievements of the team have for instance allowed dissecting the exquisite regulation of the yeast mitofusin Fzo1, a critical component of the fusion machinery of mitochondrial outer membranes, by ubiquitylation and subsequent proteasomal degradation. These studies have particular implications for deciphering the molecular basis of numerous neurodegenerative syndromes.
Miguel A. Blázquez

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Instituto de Biología Molecular y Celular de Plantas (CSIC-UPV)
http://www.ibmcp.upv.es
Spain
WG3
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Plant Molecular Biology, Biotechnology
Min Jae Lee

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Seoul National University
http://sites.google.com
Republic of Korea
WG2
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Proteasome, N-end rule pathway, Post-translational modification, Protein biochemistry, Chemical biology
Mirta Boban

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University of Zagreb School of Medicine, Croatian Institute for Brain Research
http://mef.unizg.hr
Croatia
WG4
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I am interested in protein quality control pathways in yeast Saccharomyces cerevisiae under the conditions of limited nutrient availability and the application of yeast genetics in elucidating molecular basis of protein aggregation in neurodegenerative diseases.
Mª Luz Martínez Chantar

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CIC bioGUNE
http://www.cicbiogune.es
Spain
WG6
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LIVER DISEASES. New mechanism implicated in the developing of cirrhosis and Liver Cancer
Nico Dantuma

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Karolinska Institutet
http://twitter.com
Sweden
WG1
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Developing reporter assays for ubiquitin-dependent proteolysis Understanding basic principles of the ubiquitin/proteasome system The ubiquitin/proteasome system in neurodegenerative disorders Ubiquitin signaling in the DNA damage response
Nico Dissmeyer

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Leibniz Institute of Plant Biochemistry (IPB)
http://www.dissmeyerlab.org
Germany
WG1
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Speciality: targeted protein degradation via N-end rule pathway, molecular mechanistics of target protein recognition and degradation, conditional protein accumulation in vivo (based on modulated protein degradation) Skills: protein, biochemistry, mol. biology, cell culture, and plant techniques, plant genetics, peptide synthesis on arrays, protein arginylation, cross-linking, standard mass spec in house, COFRADIC in collaboration, degradation assays, Ubiquitin fusion techniques (fusion, degradation, sandwich, etc.), protein interaction studies
Nicolas Rouhier

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UNIVERSITE DE LORRAINE
http://mycor.nancy.inra.fr
France
WG4
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We are studying redox post-translational modifications of proteins which are important for many if not all biological processes. The studied processes include the oxidative protein folding, the detoxification processes, the response to stress including pathogens, and the senescence to cite only those particularly related to PROTEOSTASIS
Nicolas Thomä

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Friedrich Miescher Institute for Biomedical Researc
http://www.fmi.ch
Switzerland
WG2
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Structure and function studies of large macromolecular assemblies involved in ubiquitination and genome maintenance
Niki Chondrogianni

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National Hellenic Research Foundation
No website
Greece
WG2
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1. Proteasome expression and function during the progression of aging in vitro (replicative senescence model), in vivo (human cohorts and C. elegans) and in age-related diseases. 2. Nrf2 regulation and function Cell culture, C. elegans cultures, Bacterial cultures, Conventional and Real-Time PCR, Cloning, Transformation, Transfection, 1D and 2D protein electrophoresis, Western blot, Immunoprecipitation, Gel filtration analysis, Microscopy, Proteasome activities assay, in-gel assays, Native gel electrophoresis, Oxidized proteins assay, Reactive Oxygen Species (ROS) detection, Lifespan assays in cell cultures and cultures of C. elegans
Noam Zelcer

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Academic Medical Center of the University of Amsterdam
http://www.zelcerlab.eu
Netherlands
WG6
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My work centers on the transcriptional and post-transcriptional regulation of lipid metabolism. A special focus is put on the role of the UPS herein and its involvement in cardiovascular and metabolic diseases.
Núria Sánchez Coll

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Centre for Research in Agricultural Genomics (CRAG)
http://www.ub.edu
Spain
WG4
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Speciality:My work focuses on the mechanisms that control programmed cell death / cell survival in plants, with particular emphasis on pathogen-triggered processes. We are studying a family of death proteases, termed metacaspases that control this processes. Recently we have found that metacaspase 1, one of the main regulators of programmed cell death has an additional role in aggregate clearance. This role is developmentally regulated and it acts in parallel to autophagy in controlling timely aging of plant cells. In addition, this proteostatic function of metacaspase 1 is conserved from plants to yeast, pointing towards an ancient role in protein quality control that might have later evolved to a cell death function. We have started a project to try to mechanistically understand how metacaspase 1 switches from a cell death to a cell survival function using a multidisciplinary approach that combines proteomics, cell biology, biochemistry and molecular biology. Skills: We are experts on plant molecular biology and genetics. We have developed assays to monitor programmed cell death in plants. In addition, in the last years we have focused on dissecting the dynamic composition of plant deathosomes ?cell death complexes- using proteomics combined with biochemistry and molecular biology. We are also plant pathologists, studying plant-microbe interactions at the molecular and physiological level.
Odile Burlet- Schiltz

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Institut de Pharmacologie et de Biologie Structurale (IPBS), CNRS, Université de Toulouse
http://www.ipbs.fr
France
WG2
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We are specialized in the field of proteomics and mass spectrometry. Using these approaches, we are conducting several research projects devoted to the understanding of biological processes involved mainly in cancer and inflammation. These projects include methodological developments in proteomic strategies focused on the analysis of protein complexes, the characterization of posttranslational modifications, the analysis of low-abundance proteins, and the identification of biomarker candidates. They rely on the use of a combination of optimized biochemistry steps (fractionation, enrichment), sophisticated large-scale and targeted quantitative mass spectrometry-based proteomic analyses, and dedicated bioinformatics tools. Among these projects, we are interested in understanding the structure/activity relationships of human proteasome. To this aim we develop proteomic strategies to characterize the subunit composition of the catalytic core of the complex, to determine the distribution of the various regulatory complexes associated to the catalytic core complex, to identify proteins interacting with the proteasome, and to quantify the variations in the composition of proteasome complexes to study their dynamics in different cells and also within the cell in different subcellular compartments.
Olivier Coux

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CRBM (Macromolecular Biochemistry Research Centre). CNRS. UMR 5238
http://www.crbm.cnrs.fr
France
WG2
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Cell biology and biochemical approaches to dissect many aspects of the specificity and mechanism of action of the ubiquitin-proteasome system, in the context of cell cycle
Olivier Vincent

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Instituto de Investigaciones Biomédicas CSIC-UAM
http://www.iib.uam.es
Spain
WG3
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Function of alpha-arrestins as endocytic adaptors involved in ubiquitinaiton processes, and role of these proteins as downstream effectors of nutrient signalling pathways / molecular basis of ubiquitin chain synthesis
Olivier Walker

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Institut des Sciences Analytiques. UMR 528-Université Lyon1
http://olivier-walker.univ-lyon1.fr
France
WG3
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We are specialized in biomolecular Nuclear Magnetic Resonance and more particularly we are focusing on all the aspect of protein/protein and protein/ligand interactions from a structural and dynamical perspective. Our current research topics focus on the interaction network involved in the endocytic pathway as well as coupled monoubiquitination from an atomic point of view.
Panagiotis Moschou

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Swedish University of Agricultural Sciences (SLU)
http://www.slu.se
Sweden
WG5
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Our research is focused on the role of catabolic pathways such as RNA degradation and proteolysis, particularly in relation to cell development and stress responses. We pursue multilevel and multidisciplinary approaches, ranging from the organismal level down to single molecules.
Pascal Genschik

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Institut de Biologie Moléculaire des Plantes - UPR2357
http://www.ibmp.cnrs.fr
France
WG2
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The main interest of my group is to unravel the role of cullin RING ligases in specific pathways, such as the cell cycle control, phytohormone signalling, genome stability and post-transcriptional gene silencing
Pascale Bomont

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INM-INSERM U151. Hospital St Eloi
http://www.inmfrance.com
France
WG2
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Our research program aims to address the direct role of the Ubiquitin Proteasome System (UPS) in neurodegeneration. For that purpose, we focus on Giant Axonal Neuropathy (GAN), a fatale neurodegenerative disorder caused by recessive mutations in gigaxonin, the substrate adaptor of a Cul3-
Pascual Sanz Bigorra

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Instituto de Biomedicina de Valencia (CSIC)
http://www.ibv.csic.es
Spain
WG1
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Our group is interested in studying the molecular basis of progressive myoclonus epilepsy of Lafora type (LD). This rare fatal disease is related to mutations in genes EPM2A, encoding a glucan phosphatase named laforin, and EPM2B, encoding an E3-ubiquitin ligase named malin. We and others have described that laforin and malin form a functional complex and, at present, we are working in characterizing specific substrates of this complex. We have identified so far several of them, either related to glycogen homeostasis (since a hallmark of the disease is the accumulation of poorly branched polyglucosans in the neurons) or related to other physiological pathways (ER-stress, proteasome function, autophagy pathway). We have described that the laforin-malin complex produce K63-linked polyubiquitin chains in the corresponding substrates and we are now defining the combination of E2-conjugases that are involved in this process. We have at hand a mouse model lacking laforin and another lacking malin. We are using these mice models to continuing performing pathophysiological studies. The results we have obtained so far have allowed us to select different compounds that we are using right now to check whether they alleviate their phenotype.
Patrice Codogno

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Institut Necker Enfants-Malades (INEM). INSERM U1151 CNRS UMR8253
http://www.institut-necker-enfants-malades.fr
France
WG2
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Speciality: Regulation of autophagy and membrane dynamics during autophagosome formation. Role of autophagy in stress response (flow and shear stress, drugs). Role of autophagy in cancer stem cells. Skills: Biochemistry of proteins. Light microscopy and confocal microscopy. Isolation of cancer stem cells. Analysis of shear stress.
Patricia Pérez-Galán

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Consorci Institut D'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS)
http://www.idibaps.org
Spain
WG6
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Aggressive B-cell lymphomas are clinically and pathologically diverse and reflect multiple pathways of transformation. Alterations in oncogenes like MYC, BCL2, and CCND1/BCL1 play a key role in the progression of the malignant clone and correlate with a high failure rate in treatment protocols, especially in "double hit" cases with translocation of two oncogenes. However, recent studies are pointing to signals related to the lymphoid microenvironment as the real determinants of this process. For the design of new therapies more selective and more suited to the biology of these lymphomas, this project focuses on the characterization of new cancer drugs able to interfere specifically with c-myc, Bcl-2 or cyclin D1, in in vitro and in vivo models of diffuse large B cell lymphoma (DLBCL), mantle cell lymphoma (MCL) and "double hit" lymphomas. We will value especially the therapeutic potential of these inhibitors in combination with conventional therapy (R-CHOP, Velcade), and experimental therapies against tumour microenvironment or based on the activation of the apoptosis program. The main research areas include the analysis of therapeutic compounds developed and validated by recognized pharmaceutical companies and academic groups, and the molecular and genetic characterization of the factors that determine the efficacy and safety of each treatment, in a set of MCL and DLBCL primary samples, a panel of 20 cell lines representative of each entity, and in a transgenic, systemic or xenotransplant mouse models of MCL, DLBCL and "double hit" lymphomas.In addition we are also interested in the role of microenvironment (mainly in the lymph nodes and bone marrow) as a source of survival signalling and as cause of relapses and resistance to therapy in Follicular Lymphoma (FL) and MCL. We are dissecting the pathways underlying tumor- stroma cell interactions. The accompanying cells we are working with are mesenquimal stromal cells (MSCs) , follicular dendrytic cells (FDCs) and macrophages. We have set up good in vitro and in vivo mouse models. The final aim is to design therapies or combination of them that abrogate this signalling and improve patient outcome. In this area we are also working in close collaboration with pharma companies, in order to test compounds that are already in preclinical phase for any disease, speeding up the bench-bedside process in our models of study. Finally, we are also interested in new generation antibodies to improve current immunotherapy approaches. Currently, we are investigating the efficacy of an anti-CD38 antibody in vitro and in vivo, in the models of MCL, FL and Chronic Lymphocytic Leukemia (CLL)
Paul Ko Ferrigno

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Avacta Life Sciences, Ltd
http://avacta.com
United Kingdom
WG1
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Affimer technology was developed as a way to fill gaps in the biochemist's/cell biologist's toolkit. Antibodies are often not available against 'difficult targets', or cannot be used for some applications eg inhibiting protein interactions in cells. Affimer proteins can be used for in vitro protein detection and characterization as well as to inhibit (or activate) protein interactions in transfected cells. A key benefit of the Affimer technology is our use of in vitro screening (as opposed to immunization for the production of antibodies). In vitro screening means that specificity can be built into the selection process, for example by counter-selecting against a wild type protein if a mutant-specific binder is required, or selecting for binders against a specific post-translationally modified protein isoform. This has allowed us to make binders that may be of specific interest to the PROTEOSTASIS groups, such as K6-ubiquitin specific binders.
Paula Cristina da Costa Alves Monteiro Ludovico

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University of Minho. Braga. Portugal
http://www.icvs.uminho.pt
Portugal
WG2
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Speciality: My current research interests include the study of cellular proteolytic systems, proteasome and autophagy and cell death in different eukaryotic cellular models. Emerging studies have pointed autophagy as a contributor to the determination of cell fate, by differentially affecting apoptosis and necrosis and therefore, this is an important aspect that has been addressed in my laboratory. Given that aging is a physiological scenario of cell death, the relationship between cell death and autophagy regulation during aging is also of particular interest. To get new insights on these aspects, different cellular models are being used, the yeast model (Saccharomyces cerevisiae), mammalian cell lines and primary bone marrow samples from patients with acute myeloide leukemia (AML). Regarding yeast, this is a crucial cellular model to address questions related not only to autophagy but also with molecular basis of aging. In this context, the role of proteolytic systems is studied in the context of the heterologously expression of human disease-related proteins such as ?-synuclein and aging. Regarding the hematopoietic malignancies, although the importance of autophagy in cancer is widely recognized, it is still unclear whether autophagy suppresses tumorigenesis or acts as a survival mechanism for leukemic cells. It was demonstrated that autophagy can be activated in response to a variety of therapeutic drugs and most existing drugs designed to kill neoplastic cells induce autophagy. Nevertheless, the impact of autophagy in AML cells response to therapeutic drugs is still elusive and thus this is one of our objectives. Preliminary data obtained from my laboratory (manuscript under preparation) show that both proteolytic systems, proteasome and autophagy are involved in the tumorigenic process and in response to therapy of AML cells. The integrated study of these aspects on yeast as well as in mammalian cells might produce important contributions to the biomedical research. Skills: The technical skills available in my laboratory include the use of yeast and mammalian cell lines as well as human bone marrow samples where we measure autophagy flux (western blot analysis/immunohistochemistry) and also the proteasome activities. For both proteolytic systems we have an array of primers to detect mRNA levels. All the techniques to study cell death (apoptosis versus necrosis) are also optimized for all the biological systems.
Paula Videira

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Medical Sciences Faculty. Chronic Diseases Research Center
http://cedoc.unl.pt
Portugal
WG1
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Glycobiology, Immunology and Tumour immunology / Basic and translational research
Paulo de Carvalho Pereira

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Faculty of Medical Sciences, Nova University of Lisbon- CEDOC – Research Center on Chronic Diseases
http://cedoc.unl.pt
Portugal
WG1
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The interests of our group focus on age-related cell damage and cell response to stress. We are interested in exploring the molecular mechanisms of age related retinal degeneration and on the impact of deregulation of proteostasis in retinal degeneration. We are also exploring non-canonical functions for proteolytic signaling and the the putative crosstalk between the various proteolytic systems in the cell. We want to understand how cooperation between proteolytic systems can contribute to the fine-tuning of proteostasis upon ageing and disease.
Pedro M. Domingos

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ITQB-UNL
http://www.itqb.unl.pt
Portugal

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The endoplasmic reticulum (ER) is the cell organelle where secreted and membrane proteins are synthesized and folded. This process requires the recruitment of ribosomes, translocation of the nascent peptides into the lumen of the ER, and a variety of post-translational modifications and folding events. When the folding capacity of the ER is impaired, the presence of misfolded proteins in the ER causes stress to the cell (“ER stress”) and activates a cellular response, the Unfolded Protein Response (UPR), to restore homeostasis in the ER. The UPR is mediated by several signaling pathways, which sense stress in the ER and activate a variety of cellular responses, such as, translational attenuation, to reduce protein synthesis and prevent further accumulation of unfolded proteins and the transcriptional upregulation of genes encoding ER chaperones and enzymes, to increase the folding capacity of the ER. However, in situations where ER stress is severe or prolonged, or when the cellular responses induced by UPR are not sufficient to overcome the origin of ER stress, cells can undergo programmed cell death (Apoptosis).