Could an Impairment in Local Translation of mRNAs in Glia be Contributing to Pathogenesis in ALS?

TDP-43 regulates the alternative splicing of hnRNP A1 to yield an aggregation-prone variant in amyotrophic lateral sclerosis

(2018) Jade-Emmanuelle Deshaies et al.   BRAIN

Myelin remodeling through experience-dependent oligodendrogenesis in the adult somatosensory cortex

(2018) Ethan G. Hughes et al.   NATURE NEUROSCIENCE

C9orf72-mediated ALS and FTD: multiple pathways to disease

(2018) Rubika Balendra et al.   Nature Reviews Neurology

Genetic mutations in RNA-binding proteins and their roles in ALS

(2017) Katannya Kapeli et al.   HUMAN GENETICS

Toxic PRnpoly-dipeptides encoded by theC9orf72repeat expansion block nuclear import and export

(2017) Kevin Y. Shi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Astrocytes locally translate transcripts in their peripheral processes

(2017) Kristina Sakers et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Dual Role of Microglia in ALS: Mechanisms and Therapeutic Approaches

(2017) Maria Concetta Geloso et al.   Frontiers in Aging Neuroscience

Amyotrophic lateral sclerosis patient iPSC-derived astrocytes impair autophagy via non-cell autonomous mechanisms

(2017) Martin Madill et al.   Molecular Brain

The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD

(2017) Brian D. Freibaum et al.   Frontiers in Molecular Neuroscience

Translation in astrocyte distal processes sets molecular heterogeneity at the gliovascular interface

(2017) Anne- Cécile Boulay et al.   Cell Discovery

Proteomic analysis of FUS interacting proteins provides insights into FUS function and its role in ALS

(2016) Marisa Kamelgarn et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Cytoplasmic poly-GA aggregates impair nuclear import of TDP-43 in C9orf72 ALS/FTLD

(2016) Bahram Khosravi et al.   HUMAN MOLECULAR GENETICS

C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins

(2016) Yong-Jie Zhang et al.   NATURE NEUROSCIENCE

Oligodendroglial NMDA Receptors Regulate Glucose Import and Axonal Energy Metabolism

(2016) Aiman S. Saab et al.   NEURON

Protein-RNA Networks Regulated by Normal and ALS-Associated Mutant HNRNPA2B1 in the Nervous System

(2016) Fernando J. Martinez et al.   NEURON

Oligodendrocytes contribute to motor neuron death in ALS via SOD1-dependent mechanism

(2016) Laura Ferraiuolo et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

High-throughput analyses of hnRNP H1 dissects its multi-functional aspect

(2016) Philip J. Uren et al.   RNA Biology

Dysregulated axonal RNA translation in amyotrophic lateral sclerosis

(2016) Kyota Yasuda et al.   Wiley Interdisciplinary Reviews-RNA

The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains

(2016) Erin G Conlon et al.   eLife

Phase Separation by Low Complexity Domains Promotes Stress Granule Assembly and Drives Pathological Fibrillization

(2015) Amandine Molliex et al.   CELL

ALS mutations in TLS/FUS disrupt target gene expression

(2015) Tristan H. Coady et al.   GENES & DEVELOPMENT

Drosha Inclusions Are New Components of Dipeptide-Repeat Protein Aggregates in FTLD-TDP and ALSC9orf72Expansion Cases

(2015) Sílvia Porta et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS

(2015) Ana Jovičić et al.   NATURE NEUROSCIENCE

Astrocytes in Neurodegenerative Disease: Table 1.

(2015) Hemali Phatnani et al.   Cold Spring Harbor Perspectives in Biology

Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions

(2014) Johnathan Cooper-Knock et al.   BRAIN

Dynamics of Oligodendrocyte Generation and Myelination in the Human Brain

(2014) Maggie S.Y. Yeung et al.   CELL

Transport and translation of MBP mRNA is regulated differently by distinct hnRNP proteins

(2014) J. Torvund-Jensen et al.   JOURNAL OF CELL SCIENCE

Loss of nuclear TDP-43 in amyotrophic lateral sclerosis (ALS) causes altered expression of splicing machinery and widespread dysregulation of RNA splicing in motor neurones

(2014) J. Robin Highley et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

A cytoplasmic quaking I isoform regulates the hnRNP F/H-dependent alternative splicing pathway in myelinating glia

(2014) M. D. Mandler et al.   NUCLEIC ACIDS RESEARCH

mRNP granules

(2014) J Ross Buchan   RNA Biology

Protein aggregation in amyotrophic lateral sclerosis

(2013) Anna M. Blokhuis et al.   ACTA NEUROPATHOLOGICA

Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS

(2013) Tania F. Gendron et al.   ACTA NEUROPATHOLOGICA

hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations

(2013) Kohji Mori et al.   ACTA NEUROPATHOLOGICA

The role of myelin and oligodendrocytes in axonal energy metabolism

(2013) Aiman S Saab et al.   CURRENT OPINION IN NEUROBIOLOGY

Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in rats

(2013) Jianbin Tong et al.   EMBO JOURNAL

Role of Stress Granules and RNA-Binding Proteins in Neurodegeneration: A Mini-Review

(2013) Tara Vanderweyde et al.   GERONTOLOGY

Fused in sarcoma (FUS): An oncogene goes awry in neurodegeneration

(2013) Dorothee Dormann et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

(2013) Hong Joo Kim et al.   NATURE

Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis

(2013) Shin H Kang et al.   NATURE NEUROSCIENCE

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy

(2013) A. Serio et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Hexanucleotide Repeats in ALS/FTD Form Length-Dependent RNA Foci, Sequester RNA Binding Proteins, and Are Neurotoxic

(2013) Youn-Bok Lee et al.   Cell Reports

Glycolytic oligodendrocytes maintain myelin and long-term axonal integrity

(2012) Ursula Fünfschilling et al.   NATURE

LIN28B induces neuroblastoma and enhances MYCN levels via let-7 suppression

(2012) Jan J Molenaar et al.   NATURE GENETICS

Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72

(2011) Melissa E. Murray et al.   ACTA NEUROPATHOLOGICA

Translation of myelin basic protein mRNA in oligodendrocytes is regulated by integrin activation and hnRNP-K

(2011) Lisbeth S. Laursen et al.   JOURNAL OF CELL BIOLOGY

Astrocytes from familial and sporadic ALS patients are toxic to motor neurons

(2011) Amanda M Haidet-Phillips et al.   NATURE BIOTECHNOLOGY

Transcriptome-wide Identification of RNA-Binding Protein and MicroRNA Target Sites by PAR-CLIP

(2010) Markus Hafner et al.   CELL

Accumulation of TDP-43 and α-actin in an amyotrophic lateral sclerosis patient with the K17I ANG mutation

(2009) Danielle Seilhean et al.   ACTA NEUROPATHOLOGICA

Astrocyte function and role in motor neuron disease: A future therapeutic target?

(2009) Daniel Blackburn et al.   GLIA

Non–cell autonomous influence of MeCP2-deficient glia on neuronal dendritic morphology

(2009) Nurit Ballas et al.   NATURE NEUROSCIENCE

Deletion of a splicing enhancer disrupts PLP1/DM20 ratio and myelin stability

(2008) Erming Wang et al.   EXPERIMENTAL NEUROLOGY

Activation of oligodendroglial Fyn kinase enhances translation of mRNAs transported in hnRNP A2–dependent RNA granules

(2008) Robin White et al.   JOURNAL OF CELL BIOLOGY

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis

(2008) Koji Yamanaka et al.   NATURE NEUROSCIENCE