Multimodal MRI quantification of the common neurostructural bases within the FTD-ALS continuum
Sign Up to like & getrecommendations! Published in 2018 at "Neurobiology of Aging"
DOI: 10.1016/j.neurobiolaging.2017.09.019
Abstract: The continuum hypothesis linking the behavioral variant of frontotemporal dementia (bvFTD) and amyotrophic lateral sclerosis (ALS) is supported by clinical, pathological, genetic, and neuroimaging evidence. In the present multimodal magnetic resonance study, we characterized the… read more here.
Keywords: mri quantification; als continuum; multimodal mri; ftd als ... See more keywords
ALS and frontotemporal dementia belong to a common disease spectrum.
Sign Up to like & getrecommendations! Published in 2017 at "Revue neurologique"
DOI: 10.1016/j.neurol.2017.04.001
Abstract: ALS is now understood to be a complex multisystem neurodegenerative disease because areas other than the motor cortices of the brain undergo degeneration. Frontotemporal dementia (FTD) may be associated with motor neuron disease, and the… read more here.
Keywords: frontotemporal dementia; disease; dementia belong; als frontotemporal ... See more keywords
Retrovirus reactivation in CHMP2BIntron5 models of frontotemporal dementia
Sign Up to like & getrecommendations! Published in 2020 at "Human Molecular Genetics"
DOI: 10.1093/hmg/ddaa142
Abstract: Abstract Frontotemporal dementia (FTD) is the second most prevalent form of pre-senile dementia after Alzheimer’s disease. Amyotrophic lateral sclerosis (ALS) can overlap genetically, pathologically and clinically with FTD indicating the two conditions are ends of… read more here.
Keywords: frontotemporal dementia; dementia; retrovirus reactivation; reactivation chmp2bintron5 ... See more keywords
Two FTD-ALS genes converge on the endosomal pathway to induce TDP-43 pathology and degeneration
Sign Up to like & getrecommendations! Published in 2022 at "Science"
DOI: 10.1126/science.abq7860
Abstract: Frontotemporal dementia and amyotrophic lateral sclerosis (FTD-ALS) are associated with both a repeat expansion in the C9orf72 gene and mutations in the TANK-binding kinase 1 (TBK1) gene. We found that TBK1 is phosphorylated in response… read more here.
Keywords: tdp pathology; endosomal pathway; pathology; pathway ... See more keywords
Genotyping and Plasma/Cerebrospinal Fluid Profiling of a Cohort of Frontotemporal Dementia–Amyotrophic Lateral Sclerosis Patients
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DOI: 10.3390/brainsci11091239
Abstract: Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are part of the same pathophysiological spectrum and have common genetic and cerebrospinal fluid (CSF) biomarkers. Our aim here was to identify causative gene variants in a… read more here.
Keywords: frontotemporal dementia; amyotrophic lateral; ftd als; lateral sclerosis ... See more keywords
Antisense, but not sense, repeat expanded RNAs activate PKR/eIF2α-dependent ISR in C9ORF72 FTD/ALS
Sign Up to like & getrecommendations! Published in 2023 at "eLife"
DOI: 10.7554/elife.85902
Abstract: GGGGCC (G4C2) hexanucleotide repeat expansion in the C9ORF72 gene is the most common genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). The repeat is bidirectionally transcribed and confers gain of toxicity. However,… read more here.
Keywords: pkr eif2; repeat; repeat expanded; expanded rnas ... See more keywords