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.

Simona Polo

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Fondazione IFOM Istituto FIRC di Oncologia Molecolare
http://www.ifom-ieo-campus.it
Italy
WG3
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My major contributions have been on the understanding of how Ub works in the endocytic pathway, with a specific focus at the critical interface between endocytosis/trafficking and intracellular signaling. The results that I have obtained over the past years have contributed to establish new fundamental paradigms: 1) Ubiquitin is an endocytic signal in mammalian cells: 2) Ubiquitin is a signaling molecule 3) Ubiquitin ligase E3s, are regulated by ubiquitination
Solange Desagher

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Institute of Molecular Genetics of Montpellier. UMR5535 CNRS-University of Montpellier
http://www.igmm.cnrs.fr
France
WG2
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The main goal of the group is to identify new molecular mechanisms controlling apoptosis in connection to the ubiquitin-proteasome system. We have identified a novel E3 ubiquitin-ligase, Trim17, that plays a crucial role in the triggering of apoptosis. Our results indicate that Trim17 is induced during neuronal apoptosis both in vitro and in vivo, that its expression is both necessary and sufficient for inducing neuronal death, and that its pro-apoptotic effect depends on its E3 activity. Moreover, we have demonstrated that Trim17 is an E3 ubiquitin-ligase for Mcl-1, an anti-apoptotic protein of the Bcl-2 family. Our current objectives are to elucidate the mechanisms of regulation of Trim17, to characterize its substrates and to determine its role in different models of both physiological and pathological apoptosis. We are notably studying the functional interaction between Trim17 and other proteins of the TRIM family, that constitutes a sub-group of RING-containing E3 ubiquitin-ligases. We are also studying the effect of Trim17 on the level and the activity of two different transcription factors. Interestingly, the interaction with Trim17 appeared to depend on the prior SUMOylation of one of these transcription factors.
Sonja Gisela Lorenz

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Rudolf Virchow Center for Experimental Biomedicine, University of Wuerzburg
http://virchow.uni-wuerzburg.de
Germany
WG1
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Posttranslational modifications, ubiquitin, ubiquitination enzymes, enzyme mechanism, tyrosine kinases, protein-protein interactions, structural biology
Stamatis Rigas

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Agricultural University of Athens
http://www.aua.gr
Greece
WG2
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Plant Developmental Biology
Stefan Hoth

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Universität Hamburg
http://www.biologie.uni-hamburg.de
Germany
WG2
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We have a strong focus on plasma membrane-localized ubiquitin ligases that regulate development and stress responses in plants. We investigate these proteins on the molecular level (beginning with the resolution of their structure in collaboration with DESY) and on the functional level in planta. The major function studied is in the control of cell death and senescence.
Stefan Marciniak

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University of Cambridge
http://www.cimr.cam.ac.uk
UK
WG3
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We wish to understand the cellular consequences of endoplasmic reticulum stress, in particular its effects on tissue growth and cell survival. In doing so, we hope to identify targets for the development of novel therapies. During endoplasmic reticulum stress, protein biosynthesis is initially attenuated through phosphorylation of the translation initiation factor eIF2? by the kinase PERK. Subsequent dephosphorylation of eIF2? following the induction of the phosphatase PPP1R15A (GADD34) restores protein translation. We previously discovered that this recovery of translation can contribute to the toxic effects of endoplasmic reticulum stress. This raises the exciting possibility that modulation of eIF2? phosphorylation may provide a useful target for the development of novel drugs to protect tissues from cell death. Cellular stresses frequently impair cell cycle progression, which can prejudice tissue growth. Using mammalian cell biology and Drosophila genetics we recently described a novel G2 cell cycle checkpoint initiated by translation attenuation during endoplasmic reticulum stress. This too provides potential targets for the development of new therapies.
Stefano Gastaldello

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Karolinska Institutet
http://ki.se
Sweeden
WG1
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Muscle and SUMO. SUMO is a small ubiquitin-like protein and is reversible attached to a specific lysine in a multitude of proteins. Modification by SUMO induced by internal or external
stenreiro@gmail.com

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CEDOC- Centro de Estudos de Doenças Crónicas
http://cedoc.unl.pt
Portugal

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Molecular mechanisms of neurodegeneration in Parkinson's disease, using yeast models. Molecular mechanisms of retinal neurodegeneration in diabetes and Parkinson's disease.
Steven Tregay

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FORMA Therapeutics
http://www.formatherapeutics.com
USA
WG1
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Deubiquitination, Sumoylation, E-ligases, and Epigenetic readers/writers / erasers and super-enhancers.
Stjepan Uldrijan

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Masaryk University, Faculty of Medicine
http://www.med.muni.cz
Czech Republic
WG1
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Cell cultures, including induced pluripotent stem cells. Analyses of cellular proliferation and viability. Flow cytometry. Analyses of cellular energy metabolism. Molecular cloning. DNA and RNA transfections. Gene targeting using CRISPR/Cas9 system. Protein interaction and degradation assays. Ubiquitylation assays in cells. Fluorescence microscopy. Transmission electron microscopy.
Sulev Kõks

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University of Tartu. Department of Pathophysiology
http://www.biomeditsiin.ut.ee
Estonia
WG6
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Genomics, RNAseq, animal experiments, the function of Wfs1 protein, ER stress in the inflammatory and degeneratiive diseases. We have studied psoriasis in the relation of ER stress and now work on the role of ER stress in the Parkinson disease. Available technologies are: Next generation sequencing, bioinformatics and statistics of NGS results, RNAseq, Exome seq, clinical biobank, animal center with in vivo imaging, transgenic facility.
Susana Seixas

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Genetic Diversity Unit. IPATIMUP
http://www.ipatimup.pt
Portugal
WG3
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Analysis of the genetic variation of proteolysis genes (SERPINs, WFDCs and KLKs) in both healthy and disease individuals. Experimental assessment of gene variants impact in gene expression and regulation, protein folding and molecular pathways. Comparative genomics and evolutionary studies.
Sykiotis Gerasimos

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University Hospital of Lausanne (CHUV)
http://www.chuv.ch
Switzerland
WG6
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My research focuses on the Nrf2 antioxidant response system and its protective roles in different systems and organisms, including flies, mice and humans. Nrf2 is a main convergence point of proteolytic and cell defense systems: it is not only activated by oxidative stress but it also cross-talks with the UPR through its activation by the endoplasmatic reticulum stress-responsive PERK kinase, and with the ALS through the interaction of sequestosome/p62 with the Nrf2 inhibitor Keap1. Moreover, Nrf2 controls the expression not only of antioxidant and detoxification genes but also of multiple proteasome subunits. Thus, Nrf2, which is itself degraded by proteolysis under normal conditions, coordinates the antioxidant response with the UPR and the ALS. My research will benefit from the networking opportunities within Proteostasis to launch new research projects on the relationship between Nrf2, UPR, and ALS signaling. As a practicing physician, I could also participate in clinically relevant projects launched by the Action. Conversely, Action participants will benefit from my expertise on Nrf2 signaling, including the experimental tools for its study at my disposal, which I can distribute freely.Skills: Clinical endocrinology and diabetology (adults, adolescents).Medical genetics and molecular genetic diagnostics.Molecular biology and model organism genetics (flies, mice).Drug discovery and clinical trials.Project evaluation (FP7, Marie Curie)
Sylvie Urbé

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Institute of Translational Medicine. 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.
Teresa Rinaldi

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UNIVERSITY OF ROME LA SAPIENZA DIP BIOLOGY and BIOTECHNOLOGY
http://bbcd.bio.uniroma1.it
Italy
WG2
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Speciality: mitochondria and proteasome, CSN complex in yeast. Skills: yeast genetics and cellular biology, fluorescent and confocal microscopy.
Teresa Zoladek

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Institute of Biochemistry and Biophysics Polish Academy of Sciences (IBB PAS)
http://www.ibb.waw.pl
Poland
WG2
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Molecular and cellular genetics and biology of yeast.Studies on the role of Rsp5-dependent ubiquitination of proteins in mechanisms of protein transport via endocytosis, autophagy, Golgi to ER transport.Role of Rsp5-dependent ubiquitination in actin cytoskeleton organization.Role of ubiquitination in tRNA metabolism. Regulation of Rsp5 ubiquitin ligase by phosphorylation. One goal of the newest grant is to find mechanisms which are disturbed in cells by mutations in VPS13A and VPS13B gene corresponding to human inherited diseases, Cohen syndrome and Chorea acanthocytosis, by using yeast as a model organism. Yeast Vps13 protein is involved in Golgi-to vacuole trafficking and was also found in actin cortical patches which are sites of endocytosis. Vps13 protein shows some homology to Atg2 protein involved in autophagy. Another goal is to find function of Atg2 in autophagy.
Theodora Farmaki

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CERTH-INAB
http://inab.certh.gr
Greece
WG2
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1. Study of the role of FKBP chaperones in seed germination under stress conditions (especially salinity). 2. Involvement of FKBP chaperones in autophagy, proteosome and SUMOylation. 3. FKBP interaction with PI3,5P2 and PI3P involved in autophagosome formation. 4. Role of phospholipases in cotton resistance to cold stress and wounding.
Thimo Kurz

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Scottish Institute for Cell Signaling. University of Dundee. SCILLS
http://www.ppu.mrc.ac.uk
United Kingdom
WG1
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My lab focuses on various aspects of the ubiquitin/UBL system. We?re specifically interested in the UBL NEDD8 and how it regulates the largest class of Ubiquitin-ligases, the Cullin-RING E3s. We have also a focus on the role of the ubiquitin system in disease. In particular, we?ve recently focused on the role of a CUL3-based CRL in blood pressure regulation and on the involvement of the ubiquitin-receptor UBQLN2 in neurodegenerative diseases.
Thomas Sommer

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Max-Delbrück-Center for Molecular Medicine and Humboldt-University Berlin
http://www.mdc-berlin.de
Germany
WG1
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Ubiquitin Proteasome Pathway, Protein Quality Control, ERAD, Membrane Protein Complexes
Thorsten Hoppe

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Institute for Genetics and CECAD Cluster of Excellence
http://www.cecad.uni-koeln.de
Germany
WG4
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Speciality:The maintenance of protein homeostasis, or proteostasis, involves the degradation of misfolded and damaged proteins and is essential for cellular function, organismal growth, and ultimately viability. The integrity of the proteome is a long-term challenge not only for individual cells but also for entire organisms since damaged and aggregated proteins accumulate with stress and aging. The ubiquitin/proteasome system (UPS) and autophagy are the major proteolytic routes embedded in a cellular quality control network that ensure efficient turnover of defective proteins. Research in my lab aims to understand proteolytic networks -especially ubiquitin based - in aging and disease. Current projects address proteostasis mechanisms focused on genome stability, protein aggregation diseases and lifespan regulation. Skills: Use of Caenorhabditis elegans as a multicellular organism to study the physiological relevance of proteostasis mechanisms. Techniques include genetic approaches, forward genetic screens, reverse genetics, phenotypic analysis including life span, protein aggregation, muscle degeneration, neuronal function, in vivo degradation assays, biochemistry, time lapse microscopy, in vivo localization, next generation sequencing of genomic DNA, mRNAs, microRNAs.
Thorsten Pfirrmann

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Institute of Physiological Chemistry
http://www.medizin.uni-halle.de
Germany
WG1
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We are mostly interested in UPS components involved in human genetic disease and in early development of Xenopus laevis. My focus lies on Ube3a, Otud3 and the CTLH complex. Additionally, we are studying the impact of protein glycation on proteostasis.
Tiago Fleming Outeiro

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University Medical Center Goettingen (UMG)
http://www.med.uni-goettingen.de
Germany
WG1
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In our work we focus on the study of protein misfolding and aggregation and how this relates to neurodegenerative diseases such as Alzheimer?s, Parkinson?s, or Huntington?s disease. We use a variety of model organisms, from yeast to mouse models, and we employ cell and molecular biology techniques applied to neuroscience. We hold expertise in fluorescence microscopy (high-content imaging, FRAP, STED), molecular biology, lentiviral vector production, primary neuronal cultures, mouse models, protein purification, aggregation assays.
Tijana Stankovic

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Institute for Biological Research “Sinisa Stankovic”
http://www.ibiss.bg.ac.rs
Serbia

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RNA and DNA isolation
Tom Nicholson

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VIVA Bioscience Ltd.
http://www.vivabioscience.com
United Kingdom

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Tommer Ravid

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The Hebrew University of Jerusalem
http://www.bio.huji.ac.il
Israel
WG4
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Speciality: Protein quality control by the ubiquitin-proteasome system. Skills: We use yeast as a model organism, employing a large number of genetic and molecular biology tools. We also employ biochemical and cell biology assays for studying misfolded protein ubiquitylation and degradation, both in vitro and in vivo. We also take a high throughput approach for studying the biochemical properties of misfolded proteins.
Tor Erik Rusten

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Institute for Cancer Research, The Norwegian Radium Hospital, Oslo University Hospital
http://www.ous-research.no
Norway
WG6
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Autophagy, Drosophila genetics, human organoid Caco2 culture, Receptor Tyrosine Kinase signaling, phosphatidylinositol kinase signaling, growth control.
Ugo Mayor

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UPV/EHU - University of the Basque Country
http://www.ugomayor.com
Spain
WG6
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Ubiquitination has essential roles in neuronal development, function and disease. Isolation of neuronal ubiquitin conjugates from living organisms has however proven difficult, but we have developed some novel strategies to overcome this.
Ulrich auf dem Keller

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ETH Zurich
http://www.mhs.biol.ethz.ch
Switzerland
WG6
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protease proteomics in tissue repair and regeneration; degradomics method development; Terminal Amine Isotopic Labeling of Substrates Skills: proteomics; degradomics Terminal Amine Isotopic Labeling of Substrates skin cell culture models animal models of wound healing and skin carcinogenesis molecular biology and cell biology
Ute Hoecker

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Botanical Institute Cluster of Excellence on Plant Sciences (CEPLAS)
http://www.botanik.uni-koeln.de
Germany
WG3
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Role of ubiquitination in responses of the plant Arabidopsis thaliana to light (light signal transduction). Study of the E3 ubiquitin ligase COP1/SPA.
Valérie Doye

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Institut Jacques Monod - CNRS - Université Paris Diderot
http://www.ijm.fr
France
WG1
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Our main research focus is the understanding of non-conventional functions of nuclear pore complexes in cell cycle, differentiation and nuclear metabolism. In particular, we are interested in the functional connexions between nuclear pores functions (in particular mRNP export) and SUMOylation.
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