User profiles for Clotilde Lagier-Tourenne

Clotilde Lagier-Tourenne

Massachusetts General Hospital and Harvard Medical School
Verified email at mgh.harvard.edu
Cited by 14685

TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration

C Lagier-Tourenne, M Polymenidou… - Human molecular …, 2010 - academic.oup.com
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are
neurodegenerative diseases with clinical and pathological overlap. Landmark discoveries of …

Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43

M Polymenidou, C Lagier-Tourenne, KR Hutt… - Nature …, 2011 - nature.com
We used cross-linking and immunoprecipitation coupled with high-throughput sequencing
to identify binding sites in 6,304 genes as the brain RNA targets for TDP-43, an RNA binding …

Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways

…, RH Baloh, S Appel, E Simpson, C Lagier-Tourenne… - Science, 2015 - science.org
Amyotrophic lateral sclerosis (ALS) is a devastating neurological disease with no effective
treatment. We report the results of a moderate-scale sequencing study aimed at increasing the …

Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs

C Lagier-Tourenne, M Polymenidou, KR Hutt… - Nature …, 2012 - nature.com
FUS/TLS (fused in sarcoma/translocated in liposarcoma) and TDP-43 are integrally involved
in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia. We found that FUS/TLS …

[HTML][HTML] Rethinking als: The fus about tdp-43

C Lagier-Tourenne, DW Cleveland - Cell, 2009 - cell.com
Mutations in TDP-43, a DNA/RNA-binding protein, cause an inherited form of the neurodegenerative
disease amyotrophic lateral sclerosis (ALS). Two recent studies (Kwiatkowski et al., …

Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration

C Lagier-Tourenne, M Baughn, F Rigo… - Proceedings of the …, 2013 - National Acad Sciences
Expanded hexanucleotide repeats in the chromosome 9 open reading frame 72 (C9orf72)
gene are the most common genetic cause of ALS and frontotemporal degeneration (FTD). …

[PDF][PDF] Gain of toxicity from ALS/FTD-linked repeat expansions in C9ORF72 is alleviated by antisense oligonucleotides targeting GGGGCC-containing RNAs

…, J Ravits, F Rigo, DW Cleveland, C Lagier-Tourenne - Neuron, 2016 - cell.com
Hexanucleotide expansions in C9ORF72 are the most frequent genetic cause of amyotrophic
lateral sclerosis and frontotemporal dementia. Disease mechanisms were evaluated in …

ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS

…, JS Han, C Lagier-Tourenne… - Proceedings of the …, 2010 - National Acad Sciences
Dominant mutations in two functionally related DNA/RNA-binding proteins, trans-activating
response region (TAR) DNA-binding protein with a molecular mass of 43 KDa (TDP-43) and …

ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43

…, SC Huelga, C Lagier-Tourenne… - Proceedings of the …, 2013 - National Acad Sciences
Transactivating response region DNA binding protein (TDP-43) is the major protein
component of ubiquitinated inclusions found in amyotrophic lateral sclerosis (ALS) and …

Animal models of neurodegenerative diseases

TM Dawson, TE Golde, C Lagier-Tourenne - Nature neuroscience, 2018 - nature.com
Animal models of adult-onset neurodegenerative diseases have enhanced the
understanding of the molecular pathogenesis of Alzheimer’s disease, Parkinson’s disease, …