Nuclear localized C9orf72-associated arginine-containing dipeptides exhibit age-dependent toxicity in C. elegans

Glycine-alanine dipeptide repeat protein contributes to toxicity in a zebrafish model of C9orf72 associated neurodegeneration

(2017) Yu Ohki et al.   Molecular Neurodegeneration

C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles

(2016) Kyung-Ha Lee et al.   CELL

Toxic PR Poly-Dipeptides Encoded by the C9orf72 Repeat Expansion Target LC Domain Polymers

(2016) Yi Lin et al.   CELL

Spt4 selectively regulates the expression of C9orf72 sense and antisense mutant transcripts

(2016) N. J. Kramer et al.   SCIENCE

OASIS 2: online application for survival analysis 2 with features for the analysis of maximal lifespan and healthspan in aging research

(2016) Seong Kyu Han et al.   Oncotarget

Forward Genetic Screen in Caenorhabditis elegans Suggests F57A10.2 and acp-4 As Suppressors of C9ORF72 Related Phenotypes

(2016) Xin Wang et al.   Frontiers in Molecular Neuroscience

Distinct C9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity

(2016) Brittany N. Flores et al.   PLoS One

Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD

(2016) Steven Boeynaems et al.   Scientific Reports

The Progranulin Cleavage Products, Granulins, Exacerbate TDP-43 Toxicity and Increase TDP-43 Levels

(2015) D. A. Salazar et al.   JOURNAL OF NEUROSCIENCE

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

(2015) Ke Zhang et al.   NATURE

GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport

(2015) Brian D. Freibaum et al.   NATURE

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

(2015) Ana Jovičić et al.   NATURE NEUROSCIENCE

RAN Translation in Huntington Disease

(2015) Monica Bañez-Coronel et al.   NEURON

Repeat associated non-ATG (RAN) translation: new starts in microsatellite expansion disorders

(2014) John Douglas Cleary et al.   CURRENT OPINION IN GENETICS & DEVELOPMENT

TDP-43 Toxicity Proceeds via Calcium Dysregulation and Necrosis in Aging Caenorhabditis elegans Motor Neurons

(2014) D. Aggad et al.   JOURNAL OF NEUROSCIENCE

Discovery of a Biomarker and Lead Small Molecules to Target r(GGGGCC)-Associated Defects in c9FTD/ALS

(2014) Zhaoming Su et al.   NEURON

Antisense Proline-Arginine RAN Dipeptides Linked to C9ORF72-ALS/FTD Form Toxic Nuclear Aggregates that Initiate In Vitro and In Vivo Neuronal Death

(2014) Xinmei Wen et al.   NEURON

C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins

(2014) S. Mizielinska et al.   SCIENCE

Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cells

(2014) I. Kwon et al.   SCIENCE

An RNAi-Based Suppressor Screen Identifies Interactors of the Myt1 Ortholog of Caenorhabditis elegans

(2014) Anna K. Allen et al.   G3-Genes Genomes Genetics

Brain distribution of dipeptide repeat proteins in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72

(2014) Yvonne S Davidson et al.   Acta Neuropathologica Communications

Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations

(2013) Ian R. Mackenzie et al.   ACTA NEUROPATHOLOGICA

Establishing a novel C. elegans model to investigate the role of autophagy in amyotrophic lateral sclerosis

(2013) Jia Li et al.   ACTA PHARMACOLOGICA SINICA

TorsinA rescues ER-associated stress and locomotive defects in C. elegans models of ALS

(2013) M. L. Thompson et al.   Disease Models & Mechanisms

Human superoxide dismutase 1 overexpression in motor neurons of Caenorhabditis elegans causes axon guidance defect and neurodegeneration

(2013) Jia Li et al.   NEUROBIOLOGY OF AGING

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

(2013) Shuo-Chien Ling et al.   NEURON

Deletion of C9ORF72 Results in Motor Neuron Degeneration and Stress Sensitivity in C. elegans

(2013) Martine Therrien et al.   PLoS One

RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia

(2013) T. Zu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mutant TDP-43 and FUS Cause Age-Dependent Paralysis and Neurodegeneration in C. elegans

(2012) Alexandra Vaccaro et al.   PLoS One

Daf-2 Signaling Modifies Mutant SOD1 Toxicity in C. elegans

(2012) Marco Boccitto et al.   PLoS One

TDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signaling

(2011) Tao Zhang et al.   HUMAN MOLECULAR GENETICS

A randomized controlled clinical trial of growth hormone in amyotrophic lateral sclerosis: clinical, neuroimaging, and hormonal results

(2011) Francesco Saccà et al.   JOURNAL OF NEUROLOGY

Structural and physiological phenotypes of disease-linked lamin mutations in C. elegans

(2011) Erin M. Bank et al.   JOURNAL OF STRUCTURAL BIOLOGY

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

OASIS: Online Application for the Survival Analysis of Lifespan Assays Performed in Aging Research

(2011) Jae-Seong Yang et al.   PLoS One

Neurotoxic effects of TDP-43 overexpression in C. elegans

(2010) Peter E.A. Ash et al.   HUMAN MOLECULAR GENETICS

Phosphorylation Promotes Neurotoxicity in a Caenorhabditis elegans Model of TDP-43 Proteinopathy

(2010) N. F. Liachko et al.   JOURNAL OF NEUROSCIENCE

Coenzyme Q protects Caenorhabditis elegans GABA neurons from calcium-dependent degeneration

(2010) L. R. Earls et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

SUBCUTANEOUS IGF-1 IS NOT BENEFICIAL IN 2-YEAR ALS TRIAL

(2009) C. L. Howe et al.   NEUROLOGY

An ALS-Linked Mutant SOD1 Produces a Locomotor Defect Associated with Aggregation and Synaptic Dysfunction When Expressed in Neurons of Caenorhabditis elegans

(2009) Jiou Wang et al.   PLoS Genetics

Destabilizing Protein Polymorphisms in the Genetic Background Direct Phenotypic Expression of Mutant SOD1 Toxicity

(2009) Tali Gidalevitz et al.   PLoS Genetics

Direct Inhibition of the Longevity-Promoting Factor SKN-1 by Insulin-like Signaling in C. elegans

(2008) Jennifer M.A. Tullet et al.   CELL

Subcutaneous IGF-1 is not beneficial in 2-year ALS trial

(2008) E. J. Sorenson et al.   NEUROLOGY