The amyloid cascade hypothesis: an updated critical review
Results from recent clinical trials of antibodies that target amyloid-β (Aβ) for Alzheimer's
disease have created excitement and have been heralded as corroboration of the amyloid …
disease have created excitement and have been heralded as corroboration of the amyloid …
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 …
neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and …
Truncated stathmin-2 is a marker of TDP-43 pathology in frontotemporal dementia
M Prudencio, J Humphrey, S Pickles… - The Journal of …, 2020 - Am Soc Clin Investig
No treatment for frontotemporal dementia (FTD), the second most common type of early-
onset dementia, is available, but therapeutics are being investigated to target the 2 main …
onset dementia, is available, but therapeutics are being investigated to target the 2 main …
[PDF][PDF] Strikingly different clinicopathological phenotypes determined by progranulin-mutation dosage
KR Smith, J Damiano, S Franceschetti… - The American Journal of …, 2012 - cell.com
We performed hypothesis-free linkage analysis and exome sequencing in a family with two
siblings who had neuronal ceroid lipofuscinosis (NCL). Two linkage peaks with maximum …
siblings who had neuronal ceroid lipofuscinosis (NCL). Two linkage peaks with maximum …
Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a review
Frontotemporal dementia (FTD) is the second most common young-onset dementia and is
clinically characterised by progressive behavioural change, executive dysfunction and …
clinically characterised by progressive behavioural change, executive dysfunction and …
The lysosomal function of progranulin, a guardian against neurodegeneration
Progranulin (PGRN), encoded by the GRN gene in humans, is a secreted growth factor
implicated in a multitude of processes ranging from regulation of inflammation to wound …
implicated in a multitude of processes ranging from regulation of inflammation to wound …
p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD …
S Al-Sarraj, A King, C Troakes, B Smith… - Acta …, 2011 - Springer
Neuronal cytoplasmic inclusions (NCIs) containing phosphorylated TDP-43 (p-TDP-43) are
the pathological hallmarks of motor neuron disease/amyotrophic lateral sclerosis …
the pathological hallmarks of motor neuron disease/amyotrophic lateral sclerosis …
[PDF][PDF] Sortilin-mediated endocytosis determines levels of the frontotemporal dementia protein, progranulin
The most common inherited form of Frontotemporal Lobar Degeneration (FTLD) known
stems from Progranulin (GRN) mutation and exhibits TDP-43 plus ubiquitin aggregates …
stems from Progranulin (GRN) mutation and exhibits TDP-43 plus ubiquitin aggregates …
Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration
The aim of this study was to improve the neuropathologic recognition and provide criteria for
the pathological diagnosis in the neurodegenerative diseases grouped as frontotemporal …
the pathological diagnosis in the neurodegenerative diseases grouped as frontotemporal …
Neuropathological background of phenotypical variability in frontotemporal dementia
KA Josephs, JR Hodges, JS Snowden… - Acta …, 2011 - Springer
Frontotemporal lobar degeneration (FTLD) is the umbrella term encompassing a
heterogeneous group of pathological disorders. With recent discoveries, the FTLDs have …
heterogeneous group of pathological disorders. With recent discoveries, the FTLDs have …