Literature DB >> 30914480

Matter over mind: Liquid phase separation and neurodegeneration.

Shana Elbaum-Garfinkle1,2.   

Abstract

Phase separation of biomolecules leading to the formation of assemblies with distinct material properties has recently emerged as a new paradigm underlying subcellular organization. The discovery that disordered proteins, long associated with aggregation in neurodegenerative disease, are also implicated in driving liquid phase separation has galvanized significant interest in exploring the relationship between misregulated phase transitions and disease. This review summarizes recent work linking liquid phase separation to neurodegeneration, highlighting a pathological role for altered phase behavior and material properties of proteins assembled via liquid phase separation. The techniques that recent and current work in this area have deployed are also discussed, as is the potential for these discoveries to promote new research directions for investigating the molecular etiologies of neurodegenerative diseases.
© 2019 Elbaum-Garfinkle.

Entities:  

Keywords:  Alzheimer disease; Parkinson disease; amyloid; amyotrophic lateral sclerosis (ALS) (Lou Gehrig disease); biomaterials; intrinsically disordered protein; material properties; neurodegeneration; phase separation; protein aggregation

Mesh:

Substances:

Year:  2019        PMID: 30914480      PMCID: PMC6509495          DOI: 10.1074/jbc.REV118.001188

Source DB:  PubMed          Journal:  J Biol Chem        ISSN: 0021-9258            Impact factor:   5.157


  75 in total

1.  A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation.

Authors:  Avinash Patel; Hyun O Lee; Louise Jawerth; Shovamayee Maharana; Marcus Jahnel; Marco Y Hein; Stoyno Stoynov; Julia Mahamid; Shambaditya Saha; Titus M Franzmann; Andrej Pozniakovski; Ina Poser; Nicola Maghelli; Loic A Royer; Martin Weigert; Eugene W Myers; Stephan Grill; David Drechsel; Anthony A Hyman; Simon Alberti
Journal:  Cell       Date:  2015-08-27       Impact factor: 41.582

Review 2.  Protein misfolding, functional amyloid, and human disease.

Authors:  Fabrizio Chiti; Christopher M Dobson
Journal:  Annu Rev Biochem       Date:  2006       Impact factor: 23.643

3.  RNA transcription modulates phase transition-driven nuclear body assembly.

Authors:  Joel Berry; Stephanie C Weber; Nilesh Vaidya; Mikko Haataja; Clifford P Brangwynne
Journal:  Proc Natl Acad Sci U S A       Date:  2015-09-08       Impact factor: 11.205

Review 4.  Ageing, neurodegeneration and brain rejuvenation.

Authors:  Tony Wyss-Coray
Journal:  Nature       Date:  2016-11-10       Impact factor: 49.962

5.  Phase behaviour of disordered proteins underlying low density and high permeability of liquid organelles.

Authors:  Ming-Tzo Wei; Shana Elbaum-Garfinkle; Alex S Holehouse; Carlos Chih-Hsiung Chen; Marina Feric; Craig B Arnold; Rodney D Priestley; Rohit V Pappu; Clifford P Brangwynne
Journal:  Nat Chem       Date:  2017-06-26       Impact factor: 24.427

6.  In vivo demonstration that alpha-synuclein oligomers are toxic.

Authors:  Beate Winner; Roberto Jappelli; Samir K Maji; Paula A Desplats; Leah Boyer; Stefan Aigner; Claudia Hetzer; Thomas Loher; Marçal Vilar; Silvia Campioni; Christos Tzitzilonis; Alice Soragni; Sebastian Jessberger; Helena Mira; Antonella Consiglio; Emiley Pham; Eliezer Masliah; Fred H Gage; Roland Riek
Journal:  Proc Natl Acad Sci U S A       Date:  2011-02-15       Impact factor: 11.205

7.  Regulated Formation of an Amyloid-like Translational Repressor Governs Gametogenesis.

Authors:  Luke E Berchowitz; Greg Kabachinski; Margaret R Walker; Thomas M Carlile; Wendy V Gilbert; Thomas U Schwartz; Angelika Amon
Journal:  Cell       Date:  2015-09-24       Impact factor: 41.582

Review 8.  Protein Phase Separation: A New Phase in Cell Biology.

Authors:  Steven Boeynaems; Simon Alberti; Nicolas L Fawzi; Tanja Mittag; Magdalini Polymenidou; Frederic Rousseau; Joost Schymkowitz; James Shorter; Benjamin Wolozin; Ludo Van Den Bosch; Peter Tompa; Monika Fuxreiter
Journal:  Trends Cell Biol       Date:  2018-03-27       Impact factor: 20.808

Review 9.  The amyloid state and its association with protein misfolding diseases.

Authors:  Tuomas P J Knowles; Michele Vendruscolo; Christopher M Dobson
Journal:  Nat Rev Mol Cell Biol       Date:  2014-06       Impact factor: 94.444

10.  Phase transition of a disordered nuage protein generates environmentally responsive membraneless organelles.

Authors:  Timothy J Nott; Evangelia Petsalaki; Patrick Farber; Dylan Jervis; Eden Fussner; Anne Plochowietz; Timothy D Craggs; David P Bazett-Jones; Tony Pawson; Julie D Forman-Kay; Andrew J Baldwin
Journal:  Mol Cell       Date:  2015-03-05       Impact factor: 17.970

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  58 in total

Review 1.  Formation of biological condensates via phase separation: Characteristics, analytical methods, and physiological implications.

Authors:  Zhe Feng; Xudong Chen; Xiandeng Wu; Mingjie Zhang
Journal:  J Biol Chem       Date:  2019-08-23       Impact factor: 5.157

Review 2.  Features of molecular recognition of intrinsically disordered proteins via coupled folding and binding.

Authors:  Jing Yang; Meng Gao; Junwen Xiong; Zhengding Su; Yongqi Huang
Journal:  Protein Sci       Date:  2019-09-04       Impact factor: 6.725

3.  Driving tau into phase-separated liquid droplets.

Authors:  Martin Margittai
Journal:  J Biol Chem       Date:  2019-07-19       Impact factor: 5.157

4.  Regulatory mechanisms of tau protein fibrillation under the conditions of liquid-liquid phase separation.

Authors:  Solomiia Boyko; Krystyna Surewicz; Witold K Surewicz
Journal:  Proc Natl Acad Sci U S A       Date:  2020-12-01       Impact factor: 11.205

5.  Effects of Fish Oil Combined with Selenium and Zinc on Learning and Memory Impairment in Aging Mice and Amyloid Precursor Protein Processing.

Authors:  Chao-Xu Fu; Lin Dai; Xiu-Yuan Yuan; Yan-Ji Xu
Journal:  Biol Trace Elem Res       Date:  2020-07-14       Impact factor: 3.738

Review 6.  Phase separation of RNA-binding proteins in physiology and disease: An introduction to the JBC Reviews thematic series.

Authors:  James Shorter
Journal:  J Biol Chem       Date:  2019-04-04       Impact factor: 5.157

7.  TDP-43 and Tau Oligomers in Alzheimer's Disease, Amyotrophic Lateral Sclerosis, and Frontotemporal Dementia.

Authors:  Mauro Montalbano; Salome McAllen; Filippa Lo Cascio; Urmi Sengupta; Stephanie Garcia; Nemil Bhatt; Anna Ellsworth; Eric A Heidelman; Omar D Johnson; Samantha Doskocil; Rakez Kayed
Journal:  Neurobiol Dis       Date:  2020-10-14       Impact factor: 5.996

Review 8.  Protein phase separation: A novel therapy for cancer?

Authors:  Wei Wang; Yingqian Chen; Aixiao Xu; Minyi Cai; Ji Cao; Hong Zhu; Bo Yang; Xuejing Shao; Meidan Ying; Qiaojun He
Journal:  Br J Pharmacol       Date:  2020-09-28       Impact factor: 8.739

9.  Zinc promotes liquid-liquid phase separation of tau protein.

Authors:  Virender Singh; Ling Xu; Solomiia Boyko; Krystyna Surewicz; Witold K Surewicz
Journal:  J Biol Chem       Date:  2020-03-30       Impact factor: 5.157

10.  Glucocorticoid receptor condensates link DNA-dependent receptor dimerization and transcriptional transactivation.

Authors:  Filipp Frank; Xu Liu; Eric A Ortlund
Journal:  Proc Natl Acad Sci U S A       Date:  2021-07-27       Impact factor: 11.205

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