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 …

Immune signaling in neurodegeneration

TR Hammond, SE Marsh, B Stevens - Immunity, 2019 - cell.com
Neurodegenerative diseases of the central nervous system progressively rob patients of
their memory, motor function, and ability to perform daily tasks. Advances in genetics and …

TDP-43 loss and ALS-risk SNPs drive mis-splicing and depletion of UNC13A

AL Brown, OG Wilkins, MJ Keuss, SE Hill, M Zanovello… - Nature, 2022 - nature.com
Variants of UNC13A, a critical gene for synapse function, increase the risk of amyotrophic
lateral sclerosis and frontotemporal dementia,–, two related neurodegenerative diseases …

TDP-43 represses cryptic exon inclusion in the FTD–ALS gene UNC13A

XR Ma, M Prudencio, Y Koike, SC Vatsavayai, G Kim… - Nature, 2022 - nature.com
A hallmark pathological feature of the neurodegenerative diseases amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD) is the depletion of RNA-binding protein …

Neuroinflammation in frontotemporal dementia

F Bright, EL Werry, C Dobson-Stone, O Piguet… - Nature Reviews …, 2019 - nature.com
Frontotemporal dementia (FTD) refers to a group of progressive neurodegenerative
disorders with different pathological signatures, genetic variability and complex disease …

[HTML][HTML] Antisense Oligonucleotides for the Study and Treatment of ALS

BD Boros, KM Schoch, CJ Kreple, TM Miller - Neurotherapeutics, 2022 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by
motor neuron loss. ALS is now associated with mutations in numerous genes, many of which …

A predictive coding framework of allostatic–interoceptive overload in frontotemporal dementia

JA Migeot, CA Duran-Aniotz, CM Signorelli… - Trends in …, 2022 - cell.com
Recent allostatic–interoceptive explanations using predictive coding models propose that
efficient regulation of the body's internal milieu is necessary to correctly anticipate …

Microglial activation in the frontal cortex predicts cognitive decline in frontotemporal dementia

M Malpetti, TE Cope, D Street, PS Jones… - Brain, 2023 - academic.oup.com
Frontotemporal dementia is clinically and neuropathologically heterogeneous, but
neuroinflammation, atrophy and cognitive impairment occur in all of its principal syndromes …

TDP-43 and other hnRNPs regulate cryptic exon inclusion of a key ALS/FTD risk gene, UNC13A

Y Koike, S Pickles, V Estades Ayuso… - PLoS …, 2023 - journals.plos.org
A major function of TAR DNA-binding protein-43 (TDP-43) is to repress the inclusion of
cryptic exons during RNA splicing. One of these cryptic exons is in UNC13A, a genetic risk …

TDP-43 and inflammation: implications for amyotrophic lateral sclerosis and frontotemporal dementia

F Bright, G Chan, A van Hummel, LM Ittner… - International Journal of …, 2021 - mdpi.com
The abnormal mislocalisation and ubiquitinated protein aggregation of the TAR DNA
binding protein 43 (TDP-43) within the cytoplasm of neurons and glia in the central nervous …