Literature DB >> 29715444

Dynamic duo - FMRP and TDP-43: Regulating common targets, causing different diseases.

Diana Ferro1, Stephen Yao1, Daniela C Zarnescu2.   

Abstract

RNA binding proteins play essential roles during development and aging, and are also involved in disease pathomechanisms. RNA sequencing and omics analyses have provided a window into systems level alterations in neurological disease, and have identified RNA processing defects among notable disease mechanisms. This review focuses on two seemingly distinct neurological disorders, the RNA binding proteins they are linked to, and their newly discovered functional relationship. When deficient, Fragile X Mental Retardation Protein (FMRP) causes developmental deficits and autistic behaviors while TAR-DNA Binding Protein (TDP-43) dysregulation causes age dependent neuronal degeneration. Recent findings that FMRP and TDP-43 associate in ribonuclear protein particles and share mRNA targets in neurons highlight the critical importance of translation regulation in synaptic plasticity and provide new perspectives on neuronal vulnerability during lifespan.
Copyright © 2018 Elsevier B.V. All rights reserved.

Entities:  

Keywords:  Amyotrophic lateral sclerosis; FMRP; Fragile X syndrome; Frontotemporal dementia; RNA binding proteins; TDP-43; mRNA targets

Mesh:

Substances:

Year:  2018        PMID: 29715444      PMCID: PMC5997554          DOI: 10.1016/j.brainres.2018.04.034

Source DB:  PubMed          Journal:  Brain Res        ISSN: 0006-8993            Impact factor:   3.252


  88 in total

Review 1.  A census of human RNA-binding proteins.

Authors:  Stefanie Gerstberger; Markus Hafner; Thomas Tuschl
Journal:  Nat Rev Genet       Date:  2014-11-04       Impact factor: 53.242

2.  Phosphorylation promotes neurotoxicity in a Caenorhabditis elegans model of TDP-43 proteinopathy.

Authors:  Nicole F Liachko; Chris R Guthrie; Brian C Kraemer
Journal:  J Neurosci       Date:  2010-12-01       Impact factor: 6.167

Review 3.  Rodent models of TDP-43: recent advances.

Authors:  William Tsao; Yun Ha Jeong; Sophie Lin; Jonathan Ling; Donald L Price; Po-Min Chiang; Philip C Wong
Journal:  Brain Res       Date:  2012-05-01       Impact factor: 3.252

Review 4.  FTD and ALS: a tale of two diseases.

Authors:  R Ferrari; D Kapogiannis; E D Huey; P Momeni
Journal:  Curr Alzheimer Res       Date:  2011-05       Impact factor: 3.498

5.  Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis.

Authors:  Sami J Barmada; Gaia Skibinski; Erica Korb; Elizabeth J Rao; Jane Y Wu; Steven Finkbeiner
Journal:  J Neurosci       Date:  2010-01-13       Impact factor: 6.167

Review 6.  The fragile-X premutation: a maturing perspective.

Authors:  Paul J Hagerman; Randi J Hagerman
Journal:  Am J Hum Genet       Date:  2004-03-29       Impact factor: 11.025

7.  Compulsive, self-injurious, and autistic behavior in children and adolescents with fragile X syndrome.

Authors:  Scott S Hall; Amy A Lightbody; Allan L Reiss
Journal:  Am J Ment Retard       Date:  2008-01

8.  TDP-43 is a developmentally regulated protein essential for early embryonic development.

Authors:  Chantelle F Sephton; Shannon K Good; Stan Atkin; Colleen M Dewey; Paul Mayer; Joachim Herz; Gang Yu
Journal:  J Biol Chem       Date:  2009-12-29       Impact factor: 5.157

9.  The fragile X syndrome protein represses activity-dependent translation through CYFIP1, a new 4E-BP.

Authors:  Ilaria Napoli; Valentina Mercaldo; Pietro Pilo Boyl; Boris Eleuteri; Francesca Zalfa; Silvia De Rubeis; Daniele Di Marino; Evita Mohr; Marzia Massimi; Mattia Falconi; Walter Witke; Mauro Costa-Mattioli; Nahum Sonenberg; Tilmann Achsel; Claudia Bagni
Journal:  Cell       Date:  2008-09-19       Impact factor: 41.582

10.  Identification of genetic modifiers of TDP-43 neurotoxicity in Drosophila.

Authors:  Lihong Zhan; Keith A Hanson; Sang Hwa Kim; Apeksha Tare; Randal S Tibbetts
Journal:  PLoS One       Date:  2013-02-27       Impact factor: 3.240

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  4 in total

Review 1.  Implications of mRNA translation dysregulation for neurological disorders.

Authors:  Aya Jishi; Xin Qi; Helen C Miranda
Journal:  Semin Cell Dev Biol       Date:  2020-10-21       Impact factor: 7.499

Review 2.  DNA Damage and Repair Deficiency in ALS/FTD-Associated Neurodegeneration: From Molecular Mechanisms to Therapeutic Implication.

Authors:  Haibo Wang; Manohar Kodavati; Gavin W Britz; Muralidhar L Hegde
Journal:  Front Mol Neurosci       Date:  2021-12-16       Impact factor: 5.639

Review 3.  Unraveling the Pathways to Neuronal Homeostasis and Disease: Mechanistic Insights into the Role of RNA-Binding Proteins and Associated Factors.

Authors:  Stylianos Ravanidis; Fedon-Giasin Kattan; Epaminondas Doxakis
Journal:  Int J Mol Sci       Date:  2018-08-03       Impact factor: 5.923

Review 4.  Dysregulation of Translation in TDP-43 Proteinopathies: Deficits in the RNA Supply Chain and Local Protein Production.

Authors:  Reed T Bjork; Nicholas P Mortimore; Suvithanandhini Loganathan; Daniela C Zarnescu
Journal:  Front Neurosci       Date:  2022-03-07       Impact factor: 4.677

  4 in total

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