Frontotemporal lobar degeneration

M Grossman, WW Seeley, AL Boxer, AE Hillis… - Nature Reviews …, 2023 - nature.com
Frontotemporal lobar degeneration (FTLD) is one of the most common causes of early-onset
dementia and presents with early social–emotional–behavioural and/or language changes …

Limbic-predominant age-related TDP-43 encephalopathy (LATE): consensus working group report

PT Nelson, DW Dickson, JQ Trojanowski, CR Jack… - Brain, 2019 - academic.oup.com
We describe a recently recognized disease entity, limbic-predominant age-related TDP-43
encephalopathy (LATE). LATE neuropathological change (LATE-NC) is defined by a …

The post-GWAS era: from association to function

MD Gallagher, AS Chen-Plotkin - The American Journal of Human …, 2018 - cell.com
During the past 12 years, genome-wide association studies (GWASs) have uncovered
thousands of genetic variants that influence risk for complex human traits and diseases. Yet …

Uncovering the heterogeneity and temporal complexity of neurodegenerative diseases with Subtype and Stage Inference

AL Young, RV Marinescu, NP Oxtoby… - Nature …, 2018 - nature.com
The heterogeneity of neurodegenerative diseases is a key confound to disease
understanding and treatment development, as study cohorts typically include multiple …

Accumulation of TMEM106B C-terminal fragments in neurodegenerative disease and aging

J Perneel, M Neumann, B Heeman, S Cheung… - Acta …, 2023 - Springer
Several studies using cryogenic electron microscopy (cryo-EM) techniques recently reported
the isolation and characterization of novel protein filaments, composed of a C-terminal …

TMEM106B core deposition associates with TDP-43 pathology and is increased in risk SNP carriers for frontotemporal dementia

JD Marks, VE Ayuso, Y Carlomagno, M Yue… - Science translational …, 2024 - science.org
Genetic variation at the transmembrane protein 106B gene (TMEM106B) has been linked to
risk of frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) through an …

[HTML][HTML] Neurobiology of axonal transport defects in motor neuron diseases: Opportunities for translational research?

KJ De Vos, M Hafezparast - Neurobiology of disease, 2017 - Elsevier
Intracellular trafficking of cargoes is an essential process to maintain the structure and
function of all mammalian cell types, but especially of neurons because of their extreme …

C9orf72 expansions in frontotemporal dementia and amyotrophic lateral sclerosis

JD Rohrer, AM Isaacs, S Mizielinska, S Mead… - The Lancet …, 2015 - thelancet.com
C9orf72 hexanucleotide repeat expansions are the most common cause of familial
frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) worldwide. The …

Frontotemporal lobar degeneration: defining phenotypic diversity through personalized medicine

DJ Irwin, NJ Cairns, M Grossman, CT McMillan… - Acta …, 2015 - Springer
Frontotemporal lobar degeneration (FTLD) comprises two main classes of
neurodegenerative diseases characterized by neuronal/glial proteinaceous inclusions (ie …

The expanding biology of the C9orf72 nucleotide repeat expansion in neurodegenerative disease

AR Haeusler, CJ Donnelly, JD Rothstein - Nature Reviews …, 2016 - nature.com
A nucleotide repeat expansion (NRE) within the chromosome 9 open reading frame 72
(C9orf72) gene was the first of this type of mutation to be linked to multiple neurological …