Fragile X protein mitigates TDP-43 toxicity by remodeling RNA granules and restoring translation.

PubWeight™: 0.86‹?›

🔗 View Article (PMID 26385636)

Published in Hum Mol Genet on September 18, 2015

Authors

Alyssa N Coyne1, Shizuka B Yamada2, Bhavani Bagevalu Siddegowda2, Patricia S Estes2, Benjamin L Zaepfel2, Jeffrey S Johannesmeyer2, Donovan B Lockwood1, Linh T Pham2, Michael P Hart3, Joel A Cassel4, Brian Freibaum5, Ashley V Boehringer6, J Paul Taylor5, Allen B Reitz4, Aaron D Gitler3, Daniela C Zarnescu7

Author Affiliations

1: Department of Molecular and Cellular Biology, Department of Neuroscience.
2: Department of Molecular and Cellular Biology.
3: Department of Genetics, Stanford University School of Medicine, Stanford, CA, USA.
4: ALS Biopharma, LLC, Doylestown, PA, USA and.
5: Department of Neurobiology, St. Jude Children's Research Hospital, Memphis, TN, USA.
6: Department of Molecular and Cellular Biology, Department of Neuroscience, Department of Neurology, University of Arizona, Tucson, AZ, USA.
7: Department of Molecular and Cellular Biology, Department of Neuroscience, Department of Neurology, University of Arizona, Tucson, AZ, USA, zarnescu@email.arizona.edu.

Articles cited by this

A new mathematical model for relative quantification in real-time RT-PCR. Nucleic Acids Res (2001) 124.61

Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science (2006) 27.96

TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science (2008) 14.09

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis. Nat Genet (2008) 9.51

TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis. Lancet Neurol (2008) 5.33

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS. Nature (2010) 5.26

Cloning and characterization of a novel cellular protein, TDP-43, that binds to human immunodeficiency virus type 1 TAR DNA sequence motifs. J Virol (1995) 5.18

Rethinking ALS: the FUS about TDP-43. Cell (2009) 4.71

Drosophila fragile X-related gene regulates the MAP1B homolog Futsch to control synaptic structure and function. Cell (2001) 4.21

TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci U S A (2009) 4.18

Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron (2013) 3.63

TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity. J Biol Chem (2009) 3.29

The changing scene of amyotrophic lateral sclerosis. Nat Rev Neurosci (2013) 3.16

The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease. Brain Res (2012) 3.00

TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor. J Neurochem (2007) 2.91

Stress granules as crucibles of ALS pathogenesis. J Cell Biol (2013) 2.80

A yeast functional screen predicts new candidate ALS disease genes. Proc Natl Acad Sci U S A (2011) 2.68

TDP-43 is recruited to stress granules in conditions of oxidative insult. J Neurochem (2009) 2.57

Tar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue. PLoS One (2010) 2.49

Understanding the role of TDP-43 and FUS/TLS in ALS and beyond. Curr Opin Neurobiol (2011) 2.48

Altered ribostasis: RNA-protein granules in degenerative disorders. Cell (2013) 2.38

A Drosophila model for TDP-43 proteinopathy. Proc Natl Acad Sci U S A (2010) 2.36

Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery. J Proteome Res (2010) 2.35

Prion-like disorders: blurring the divide between transmissibility and infectivity. J Cell Sci (2010) 2.29

Regulated protein aggregation: stress granules and neurodegeneration. Mol Neurodegener (2012) 1.95

Exome-wide rare variant analysis identifies TUBA4A mutations associated with familial ALS. Neuron (2014) 1.85

Local RNA translation at the synapse and in disease. J Neurosci (2011) 1.83

TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1. Hum Mol Genet (2011) 1.75

Distinct expression patterns detected within individual tissues by the GAL4 enhancer trap technique. Genome (1996) 1.66

Frontotemporal dementia and amyotrophic lateral sclerosis-associated disease protein TDP-43 promotes dendritic branching. Mol Brain (2009) 1.65

TDP-43 is directed to stress granules by sorbitol, a novel physiological osmotic and oxidative stressor. Mol Cell Biol (2010) 1.64

Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models. Nat Genet (2013) 1.63

Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations. Neuron (2014) 1.63

TDP-43-mediated neuron loss in vivo requires RNA-binding activity. PLoS One (2010) 1.58

Prion-like properties of pathological TDP-43 aggregates from diseased brains. Cell Rep (2013) 1.49

FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosis. PLoS Genet (2011) 1.46

TDP-43 is a culprit in human neurodegeneration, and not just an innocent bystander. Mamm Genome (2008) 1.44

Phosphorylation promotes neurotoxicity in a Caenorhabditis elegans model of TDP-43 proteinopathy. J Neurosci (2010) 1.41

Endogenous TDP-43 localized to stress granules can subsequently form protein aggregates. Neurochem Int (2012) 1.28

The ALS disease protein TDP-43 is actively transported in motor neuron axons and regulates axon outgrowth. Hum Mol Genet (2012) 1.25

Progranulin is neurotrophic in vivo and protects against a mutant TDP-43 induced axonopathy. PLoS One (2010) 1.25

Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disorders. Hum Mol Genet (2013) 1.21

Wild-type and A315T mutant TDP-43 exert differential neurotoxicity in a Drosophila model of ALS. Hum Mol Genet (2011) 1.18

Fragile X protein controls the efficacy of mRNA transport in Drosophila neurons. Mol Cell Neurosci (2008) 1.18

Neurodegeneration-associated TDP-43 interacts with fragile X mental retardation protein (FMRP)/Staufen (STAU1) and regulates SIRT1 expression in neuronal cells. J Biol Chem (2012) 0.89

Fragile X Protein is required for inhibition of insulin signaling and regulates glial-dependent neuroblast reactivation in the developing brain. Brain Res (2012) 0.89

RNA binding mediates neurotoxicity in the transgenic Drosophila model of TDP-43 proteinopathy. Hum Mol Genet (2013) 0.89

Motor neurons and glia exhibit specific individualized responses to TDP-43 expression in a Drosophila model of amyotrophic lateral sclerosis. Dis Model Mech (2013) 0.88

Development of a novel nonradiometric assay for nucleic acid binding to TDP-43 suitable for high-throughput screening using AlphaScreen technology. J Biomol Screen (2010) 0.87

c9RAN translation: a potential therapeutic target for the treatment of amyotrophic lateral sclerosis and frontotemporal dementia. Expert Opin Ther Targets (2013) 0.85

Futsch/MAP1B mRNA is a translational target of TDP-43 and is neuroprotective in a Drosophila model of amyotrophic lateral sclerosis. J Neurosci (2014) 0.84

TDP-43 suppresses CGG repeat-induced neurotoxicity through interactions with HnRNP A2/B1. Hum Mol Genet (2014) 0.83

Characterization of a series of 4-aminoquinolines that stimulate caspase-7 mediated cleavage of TDP-43 and inhibit its function. Biochimie (2012) 0.82

CGG repeats in RNA modulate expression of TDP-43 in mouse and fly models of fragile X tremor ataxia syndrome. Hum Mol Genet (2014) 0.81