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

Tania F. Gendron, Kevin F. Bieniek, Yong‐Jie Zhang, +13

Acta Neuropathologica

ChromatinNeurodegenerative DiseasesRna ProcessingAmyotrophic Lateral SclerosisNatural Sciences +12

Tau Protein Disrupts Nucleocytoplasmic Transport in Alzheimer’s Disease

Bahareh Eftekharzadeh, J. Gavin Daigle, Larisa E. Kapinos, +27

Neuron

Neurodegenerative DiseasesAlzheimer's DiseaseMolecular NeuroscienceMedicineMolecular Biology +6

<i>C9ORF72</i> repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits

Jeannie Chew, Tania F. Gendron, Mercedes Prudencio, +23

Science

Mouse BrainsSynaptic SignalingSocial SciencesNeurobiology Of DiseaseExperimental Neuropathology +15

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

Zhaoming Su, Yong‐Jie Zhang, Tania F. Gendron, +25

Neuron

Amyotrophic Lateral SclerosisMolecular BiologyLead Small MoleculesMolecular DiagnosticsCell Biology

Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress

Yong‐Jie Zhang, Karen Jansen‐West, Ya-Fei Xu, +18

Acta Neuropathologica

GeneticsAggregation-prone C9ftd/als PolyMolecular BiologyEr StressDegenerative Pathology +15

Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis

Yong‐Jie Zhang, Tania F. Gendron, Mark Ebbert, +20

Nature Medicine

Amyotrophic Lateral SclerosisAlzheimer's DiseaseDementiaProtein TranslationStress Granule Dynamics +7

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

Yong‐Jie Zhang, Tania F. Gendron, Jonathan C. Grima, +32

Nature Neuroscience

Protein FunctionSignal TransductionNucleocytoplasmic Transport ProteinsMedicineNatural Sciences +9

Poly(GP) proteins are a useful pharmacodynamic marker for <i>C9ORF72</i> -associated amyotrophic lateral sclerosis

Tania F. Gendron, Jeannie Chew, Jeannette N. Stankowski, +75

Science Translational Medicine

Heterochromatin anomalies and double-stranded RNA accumulation underlie <i>C9orf72</i> poly(PR) toxicity

Yong‐Jie Zhang, Lin Guo, Patrick Gonzales, +32

Science

Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy

Jeannie Chew, Casey Cook, Tania F. Gendron, +12

Molecular Neurodegeneration