© 2020

Proteostasis and Disease

From Basic Mechanisms to Clinics

  • Rosa Barrio
  • James D. Sutherland
  • Manuel S. Rodriguez

Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 1233)

Table of contents

  1. Front Matter
    Pages i-xii
  2. Cancer

    1. Front Matter
      Pages 1-1
    2. Beatriz Pérez-Benavente, Alihamze Fathinajafabadi Nasresfahani, Rosa Farràs
      Pages 3-28
    3. Pierre Gâtel, Marc Piechaczyk, Guillaume Bossis
      Pages 29-54
    4. Olivier Coux, Barbara A. Zieba, Silke Meiners
      Pages 55-100
    5. Leena Arpalahti, Caj Haglund, Carina I. Holmberg
      Pages 101-115
    6. Petek Ballar Kirmizibayrak, Burcu Erbaykent-Tepedelen, Oguz Gozen, Yalcin Erzurumlu
      Pages 117-151
    7. Maria Gonzalez-Santamarta, Grégoire Quinet, Diana Reyes-Garau, Brigitte Sola, Gaël Roué, Manuel S. Rodriguez
      Pages 153-174
  3. Neurodegeneration

    1. Front Matter
      Pages 175-175
    2. Katerina Papanikolopoulou, Efthimios M. C. Skoulakis
      Pages 177-194
    3. Lee D. Harris, Sarah Jasem, Julien D. F. Licchesi
      Pages 195-221
    4. Diana F. Lázaro, Tiago F. Outeiro
      Pages 223-236
  4. Infection, Inflammation and Developmental Disorders

    1. Front Matter
      Pages 261-261
    2. Ahmed El Motiam, Santiago Vidal, Rocío Seoane, Yanis H. Bouzaher, José González-Santamaría, Carmen Rivas
      Pages 263-277
    3. Daniela Batista-Almeida, Tania Martins-Marques, Teresa Ribeiro-Rodrigues, Henrique Girao
      Pages 279-302
  5. Diet

    1. Front Matter
      Pages 327-327

About this book


This book, written by members of the European network PROTEOSTASIS, provides an up-to-date review of the research regarding protein homeostasis in health and disease. With new discoveries contributing to the increasing complexity of this topic, the book offers a detailed overview of the pathways regulating protein homeostasis, including autophagy and the ubiquitin protein family. Following a basic introduction, it explains how defects in protein homeostasis contribute to numerous pathologies, including cancer, neurodegeneration, inflammation and a number of rare diseases. In addition, it discusses, the role of protein homeostasis in cellular development and physiology. 

Highlighting the latest research in the field of protein homeostasis and its implications for various clinically relevant diseases, the book appeals to researchers and clinicians, while also offering a reference guide for scholars who are new to the field.


Autophagy Cancer Development Inflammation Neurodegeneration Physiology Proteostasis Protein Homeostasis Rare Disease Ubiquitin Ubiquitin-likes

Editors and affiliations

  • Rosa Barrio
    • 1
  • James D. Sutherland
    • 2
  • Manuel S. Rodriguez
    • 3
  1. 1.CIC bioGUNE, Basque Research and Technology Alliance (BRTA)DerioSpain
  2. 2.CIC bioGUNE, Basque Research and Technology Alliance (BRTA)DerioSpain
  3. 3.Centre Pierre Potier, ITAVToulouse Cedex 1France

About the editors

Rosa Barrio obtained her PhD in the Centre of Molecular Biology Severo Ochoa (Madrid, Spain) on the study of ubiquitin genes in Drosophila. Since then, she was interested on the effect of ubiquitin-like molecules on relevant transcription factors involved in organismal development and physiology. After her postdoctoral training at European Molecular Biology Laboratory (Heidelberg, Germany), she is a principal investigator in the Functional Genomics Unit of CIC bioGUNE (Derio, Spain) since December 2004. Using Drosophila as a model system, her laboratory unveiled the role of SUMOylation in vivo in the processes of steroidogenesis through the regulation of the Steroidogenic Factor 1, and development through the regulation of the Spalt-like factors (SALL) and ciliogenesis. The laboratory developed technology for the study posttranslational modifications by members of the ubiquitin family, and apply this technology for the analysis of the ubiquitin code.

Jim Sutherland is a developmental/cellular biologist studying protein homeostasis regulation in the primary cilia. His doctoral research on USP/DHR38 heterodimers and the Drosophila ecdysone response was completed at Harvard University and EMBL under the supervision of Dr. Fotis Kafatos. His post-doctoral research was focused on the functional analysis of profilin and regulation of the actin cytoskeleton in the mouse (Witke Group, EMBL Rome) and on the role of TES in cellular adhesion (Way Group, Cancer Research UK, London). In 2005, he joined the Cooperative Research Centre bioGUNE (CIC bioGUNE) as a staff researcher. He develops novel tools for studying modifications by ubiquitin and ubiquitin-like proteins. These are applied towards understanding primary cilia formation and function, with special focus on Townes Brock Syndrome, a rare ciliopathy caused by SALL1 mutations and characterized by developmental defects and kidney dysfunction.

Manuel S Rodriguez obtained his PhD degree in microbiology at Pasteur Institute (Paris, France). Early in his carrier he became interested in the role of ubiquitin family members in the regulation of critical cellular factors such as those involved in the immune response, apoptosis or oncogenesis. He was particularly intrigued by the role of ubiquitin-like proteins (UbLs) in the regulation of protein homeostasis in situations when two or more members of this family were acting on the same protein or pathways. Some of his work was oriented to study the regulation of NF-kB and the tumour suppressor p53. More recently his efforts have been oriented to the study of the crosstalk between the ubiquitin proteasome and autophagy-Lysosome systems. His group became particularly interested in a selective autophagy mechanism named proteaphagy that appears to regulate the pool of 26S proteasome in response to proteasome inhibition. Among other contributions he developed methods to study post-modification events and its functional consequences. Including ubiquitin capturing systems (named TUBEs) based in the tandem disposition of ubiquitin binding domains and a version of SUMO-traps that have been used to study the response of the cell to distinct stimuli/infections. Manuel S. Rodriguez is currently a group leader at the Institute for advanced technologies in life sciences (ITAV) from the CNRS (Toulouse, France). 

Bibliographic information

  • Book Title Proteostasis and Disease
  • Book Subtitle From Basic Mechanisms to Clinics
  • Editors Rosa Barrio
    James D. Sutherland
    Manuel S. Rodriguez
  • Series Title Advances in Experimental Medicine and Biology
  • Series Abbreviated Title Adv Exp Med Biol
  • DOI
  • Copyright Information Springer Nature Switzerland AG 2020
  • Publisher Name Springer, Cham
  • eBook Packages Biomedical and Life Sciences Biomedical and Life Sciences (R0)
  • Hardcover ISBN 978-3-030-38265-0
  • Softcover ISBN 978-3-030-38268-1
  • eBook ISBN 978-3-030-38266-7
  • Series ISSN 0065-2598
  • Series E-ISSN 2214-8019
  • Edition Number 1
  • Number of Pages XII, 348
  • Number of Illustrations 18 b/w illustrations, 24 illustrations in colour
  • Topics Cell Biology
    Biomedicine, general
    Cancer Research
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