Author details

Full Name

Tania F Gendron

Affiliation

ORCID

Career Start Year

2000

Papers

115

H Index

Expertise

CM4AI Collaborator

PMID	Paper Title	Journal Title	Published Year
37434215	Targeting the glycine-rich domain of TDP-43 with antibodies prevents its aggregation in vitro and reduces neurofilament levels in vivo.		2023
36374516	Association of Physical Activity With Neurofilament Light Chain Trajectories in Autosomal Dominant Frontotemporal Lobar Degeneration Variant Carriers.	JAMA Neurol	2023
36100130	Race affects adverse outcomes of deep vein thrombosis, pulmonary embolism, and acute kidney injury in coronavirus disease 2019 hospitalized patients.	J Vasc Surg Venous Lymphat Disord	2023
37752346	Artificial microRNA suppresses C9ORF72 variants and decreases toxic dipeptide repeat proteins in vivo.		2023
37939160	Immunological drivers of amyotrophic lateral sclerosis.		2023
38014069	Aberrant splicing exonizes repeat expansion in ALS/FTD.		2023
37346371	Moderate intrinsic phenotypic alterations in ALS/FTD iPSC-microglia despite the presence of C9orf72 pathological features.		2023
37471224	Poly(GR) interacts with key stress granule factors promoting its assembly into cytoplasmic inclusions.	Cell Rep	2023
36711601	Single nucleus multiome analysis of the prefrontal cortex from ALS/FTD patients illuminates pathways affected during disease progression.		2023
37461319	FUS regulates RAN translation through modulating the G-quadruplex structure of GGGGCC repeat RNA in <i>C9orf72</i>-linked ALS/FTD.	Elife	2023
36454749	Comprehensive evaluation of human-derived anti-poly-GA antibodies in cellular and animal models of <i>C9orf72</i> disease.	Proc Natl Acad Sci U S A	2022
36103513	Poly(ADP-ribose) promotes toxicity of <i>C9ORF72</i> arginine-rich dipeptide repeat proteins.	Sci Transl Med	2022
35879741	Evidence of cerebellar TDP-43 loss of function in FTLD-TDP.	Acta Neuropathol Commun	2022
36213006	Neurofilament light chain and vaccination status associate with clinical outcomes in severe COVID-19.	iScience	2022
36271076	CRISPR/Cas9-mediated excision of ALS/FTD-causing hexanucleotide repeat expansion in C9ORF72 rescues major disease mechanisms in vivo and in vitro.	Nat Commun	2022
36201573	Two FTD-ALS genes converge on the endosomal pathway to induce TDP-43 pathology and degeneration.	Science	2022
36409902	A blood-brain penetrant RNA-targeted small molecule triggers elimination of r(G<sub>4</sub>C<sub>2</sub>)<sup>exp</sup> in c9ALS/FTD via the nuclear RNA exosome.	Proc Natl Acad Sci U S A	2022
34705518	Ribonuclease recruitment using a small molecule reduced c9ALS/FTD r(GC) repeat expansion in vitro and in vivo ALS models.	Science Translational Medicine	2021
33736701	Alterations of mesenchymal stromal cells in cerebrospinal fluid: insights from transcriptomics and an ALS clinical trial.	Stem Cell Research and Therapy	2021
33889947	Long-read targeted sequencing uncovers clinicopathological associations for C9orf72-linked diseases.	Brain	2021
34131052	Serum neurofilament light protein correlates with unfavorable clinical outcomes in hospitalized patients with COVID-19.	Sci Transl Med	2021
33112198	Sensitive ELISA-based detection method for the mitophagy marker p-S65-Ub in human cells, autopsy brain, and blood samples.	Autophagy	2021
34677935	A Small Molecule Exploits Hidden Structural Features within the RNA Repeat Expansion That Causes c9ALS/FTD and Rescues Pathological Hallmarks.	ACS Chemical Neuroscience	2021
34654821	A C. elegans model of C9orf72-associated ALS/FTD uncovers a conserved role for eIF2D in RAN translation.	Nature Communications	2021
32790644	Truncated stathmin-2 is a marker of TDP-43 pathology in frontotemporal dementia.	J Clin Invest	2020
32375063	Chimeric Peptide Species Contribute to Divergent Dipeptide Repeat Pathology in c9ALS/FTD and SCA36.	Neuron	2020
32375043	Hexanucleotide Repeat Expansions in c9FTD/ALS and SCA36 Confer Selective Patterns of Neurodegeneration InÂ Vivo.	Cell Rep	2020
32059759	Nucleocytoplasmic Proteomic Analysis Uncovers eRF1 and Nonsense-Mediated Decay as Modifiers of ALS/FTD C9orf72 Toxicity.	Neuron	2020
31937582	Cross-sectional and longitudinal measures of chitinase proteins in amyotrophic lateral sclerosis and expression of CHI3L1 in activated astrocytes.	Journal of Neurology, Neurosurgery and Psychiatry	2020
32284607	Reduced C9ORF72 function exacerbates gain of toxicity from ALS/FTD-causing repeat expansion in C9orf72.	Nat Neurosci	2020
32093728	Dipeptide repeat proteins inhibit homology-directed DNA double strand break repair in C9ORF72 ALS/FTD.	Molecular Neurodegeneration	2020
32878979	<i>C9orf72</i> poly(GR) aggregation induces TDP-43 proteinopathy.	Sci Transl Med	2020
33196168	Structural Features of Small Molecules Targeting the RNA Repeat Expansion That Causes Genetically Defined ALS/FTD.	ACS Chemical Biology	2020
33087504	Toward allele-specific targeting therapy and pharmacodynamic marker for spinocerebellar ataxia type 3.	Sci Transl Med	2020
33177179	Plasma neurofilament light predicts mortality in patients with stroke.	Sci Transl Med	2020
31358992	RPS25 is required for efficient RAN translation of C9orf72 and other neurodegenerative disease-associated nucleotide repeats.	Nat Neurosci	2019
31594549	Extensive transcriptomic study emphasizes importance of vesicular transport in C9orf72 expansion carriers.	Acta Neuropathol Commun	2019
31784536	Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD.	Nature Communications	2019
30503283	The Hairpin Form of r(GC) in c9ALS/FTD Is Repeat-Associated Non-ATG Translated and a Target for Bioactive Small Molecules.	Cell Chemical Biology	2019
30767771	Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy.	Mol Neurodegener	2019
30765536	Heterochromatin anomalies and double-stranded RNA accumulation underlie <i>C9orf72</i> poly(PR) toxicity.	Science	2019
28130314	Disease Mechanisms of Repeat Expansions.	Cold Spring Harbor Perspectives in Medicine	2018
30029693	Poly-GR dipeptide repeat polymers correlate with neurodegeneration and Clinicopathological subtypes in C9ORF72-related brain disease.	Acta Neuropathol Commun	2018
30401437	Amyotrophic Lateral Sclerosis: An Update for 2018.	Mayo Clinic Proceedings	2018
30568632	Biomarkers for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Associated With Hexanucleotide Expansion Mutations in .	Frontiers in Neurology	2018
29855382	Loss of Tmem106b is unable to ameliorate frontotemporal dementia-like phenotypes in an AAV mouse model of C9ORF72-repeat induced toxicity.	Acta Neuropathol Commun	2018
29942091	Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis.	Nat Med	2018
30126445	Long-read sequencing across the C9orf72 'GGGGCC' repeat expansion: implications for clinical use and genetic discovery efforts in human disease.	Mol Neurodegener	2018
29761121	Poly(GP), neurofilament and grey matter deficits in <i>C9orf72</i> expansion carriers.	Ann Clin Transl Neurol	2018
29302778	A zebrafish model for C9orf72 ALS reveals RNA toxicity as a pathogenic mechanism.	Acta Neuropathologica	2018