[HTML][HTML] Lysosome dysfunction as a cause of neurodegenerative diseases: Lessons from frontotemporal dementia and amyotrophic lateral sclerosis

J Root, P Merino, A Nuckols, M Johnson… - Neurobiology of disease, 2021 - Elsevier
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are fatal
neurodegenerative disorders that are thought to exist on a clinical and pathological …

TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia

IRA Mackenzie, R Rademakers… - The Lancet Neurology, 2010 - thelancet.com
Abnormal intracellular protein aggregates comprise a key characteristic in most
neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and …

[PDF][PDF] Progranulin deficiency promotes circuit-specific synaptic pruning by microglia via complement activation

H Lui, J Zhang, SR Makinson, MK Cahill, KW Kelley… - Cell, 2016 - cell.com
Microglia maintain homeostasis in the brain, but whether aberrant microglial activation can
cause neurodegeneration remains controversial. Here, we use transcriptome profiling to …

Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a review

H Seelaar, JD Rohrer, YAL Pijnenburg… - Journal of Neurology …, 2011 - jnnp.bmj.com
Frontotemporal dementia (FTD) is the second most common young-onset dementia and is
clinically characterised by progressive behavioural change, executive dysfunction and …

Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management

O Piguet, M Hornberger, E Mioshi… - The Lancet …, 2011 - thelancet.com
Patients with behavioural-variant frontotemporal dementia (bvFTD) present with insidious
changes in personality and interpersonal conduct that indicate progressive disintegration of …

[PDF][PDF] Sortilin-mediated endocytosis determines levels of the frontotemporal dementia protein, progranulin

F Hu, T Padukkavidana, CB Vægter, OA Brady… - Neuron, 2010 - cell.com
The most common inherited form of Frontotemporal Lobar Degeneration (FTLD) known
stems from Progranulin (GRN) mutation and exhibits TDP-43 plus ubiquitin aggregates …

Advances in understanding the molecular basis of frontotemporal dementia

R Rademakers, M Neumann… - Nature Reviews Neurology, 2012 - nature.com
Frontotemporal dementia (FTD) is a clinical syndrome with a heterogeneous molecular
basis. Until recently, the underlying cause was known in only a minority of cases that were …

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 …

Primary progressive aphasia: clinicopathological correlations

M Grossman - Nature Reviews Neurology, 2010 - nature.com
Primary progressive aphasia (PPA) is a disorder of declining language that is a frequent
presentation of neurodegenerative diseases such as frontotemporal lobar degeneration …

Frontotemporal dementia

D Neary, J Snowden, D Mann - The Lancet Neurology, 2005 - thelancet.com
Frontotemporal dementia (FTD) is a focal clinical syndrome characterised by profound
changes in personality and social conduct and associated with circumscribed degeneration …