The cerebellum and cognition

JD Schmahmann - Neuroscience letters, 2019 - Elsevier
What the cerebellum does to sensorimotor and vestibular control, it also does to cognition,
emotion, and autonomic function. This hypothesis is based on the theories of dysmetria of …

Frontotemporal dementia

J Bang, S Spina, BL Miller - The Lancet, 2015 - thelancet.com
Frontotemporal dementia is an umbrella clinical term that encompasses a group of
neurodegenerative diseases characterised by progressive deficits in behaviour, executive …

[HTML][HTML] LATE-NC staging in routine neuropathologic diagnosis: an update

PT Nelson, EB Lee, MD Cykowski, I Alafuzoff… - Acta …, 2023 - Springer
An international consensus report in 2019 recommended a classification system for limbic-
predominant age-related TDP-43 encephalopathy neuropathologic changes (LATE-NC) …

[HTML][HTML] Human ALS/FTD brain organoid slice cultures display distinct early astrocyte and targetable neuronal pathology

K Szebényi, LMD Wenger, Y Sun, AWE Dunn… - Nature …, 2021 - nature.com
Amyotrophic lateral sclerosis overlapping with frontotemporal dementia (ALS/FTD) is a fatal
and currently untreatable disease characterized by rapid cognitive decline and paralysis …

[PDF][PDF] Gain of toxicity from ALS/FTD-linked repeat expansions in C9ORF72 is alleviated by antisense oligonucleotides targeting GGGGCC-containing RNAs

J Jiang, Q Zhu, TF Gendron, S Saberi… - Neuron, 2016 - cell.com
Hexanucleotide expansions in C9ORF72 are the most frequent genetic cause of
amyotrophic lateral sclerosis and frontotemporal dementia. Disease mechanisms were …

The C9orf72 protein interacts with Rab1a and the ULK 1 complex to regulate initiation of autophagy

CP Webster, EF Smith, CS Bauer, A Moller… - The EMBO …, 2016 - embopress.org
A GGGGCC hexanucleotide repeat expansion in the C9orf72 gene is the most common
genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia (C9 ALS/FTD) …

[HTML][HTML] C9ORF72: what it is, what it does, and why it matters

J Smeyers, EG Banchi, M Latouche - Frontiers in cellular …, 2021 - frontiersin.org
When the non-coding repeat expansion in the C9ORF72 gene was discovered to be the
most frequent cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis …

Extracellular vesicles in neurodegenerative disease—pathogenesis to biomarkers

AG Thompson, E Gray, SM Heman-Ackah… - Nature Reviews …, 2016 - nature.com
To develop effective disease-modifying therapies for neurodegenerative diseases, reliable
markers of diagnosis, disease activity and progression are a research priority. The fact that …

The frontotemporal dementia-motor neuron disease continuum

JR Burrell, GM Halliday, JJ Kril, LM Ittner, J Götz… - The Lancet, 2016 - thelancet.com
Early reports of cognitive and behavioural deficits in motor neuron disease might have been
overlooked initially, but the concept of a frontotemporal dementia-motor neuron disease …

Autophagy and ALS: mechanistic insights and therapeutic implications

JP Chua, H De Calbiac, E Kabashi, SJ Barmada - Autophagy, 2022 - Taylor & Francis
Mechanisms of protein homeostasis are crucial for overseeing the clearance of misfolded
and toxic proteins over the lifetime of an organism, thereby ensuring the health of neurons …