[HTML][HTML] iPSC-based disease modeling and drug discovery in cardinal neurodegenerative disorders

H Okano, S Morimoto - Cell Stem Cell, 2022 - cell.com
It has been 15 years since the birth of human induced pluripotent stem cell (iPSC)
technology in 2007, and the scope of its application has been expanding. In addition to the …

Amyotrophic lateral sclerosis-frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria

MJ Strong, S Abrahams, LH Goldstein… - … lateral sclerosis and …, 2017 - Taylor & Francis
This article presents the revised consensus criteria for the diagnosis of frontotemporal
dysfunction in amyotrophic lateral sclerosis (ALS) based on an international research …

Mitophagy in human diseases

L Doblado, C Lueck, C Rey, AK Samhan-Arias… - International journal of …, 2021 - mdpi.com
Mitophagy is a selective autophagic process, essential for cellular homeostasis, that
eliminates dysfunctional mitochondria. Activated by inner membrane depolarization, it plays …

The hubs of the human connectome are generally implicated in the anatomy of brain disorders

NA Crossley, A Mechelli, J Scott, F Carletti, PT Fox… - Brain, 2014 - academic.oup.com
Brain networks or 'connectomes' include a minority of highly connected hub nodes that are
functionally valuable, because their topological centrality supports integrative processing …

The epidemiology of ALS: a conspiracy of genes, environment and time

A Al-Chalabi, O Hardiman - Nature Reviews Neurology, 2013 - nature.com
Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive neurodegenerative disease
of motor neurons, resulting in worsening weakness of voluntary muscles until death from …

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 …

Gene discovery in amyotrophic lateral sclerosis: implications for clinical management

A Al-Chalabi, LH Van Den Berg, J Veldink - Nature Reviews Neurology, 2017 - nature.com
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease predominantly affecting
upper and lower motor neurons. The disease leads to relentlessly progressive weakness of …

Screening for cognition and behaviour changes in ALS

S Abrahams, J Newton, E Niven, J Foley… - … lateral sclerosis and …, 2014 - Taylor & Francis
This study presents the Edinburgh Cognitive and Behavioural ALS Screen (ECAS),
developed for ALS patients with physical disability for use by health care professionals. The …

ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS

C Crockford, J Newton, K Lonergan, T Chiwera… - Neurology, 2018 - AAN Enterprises
Objective To elucidate the relationship between disease stage in amyotrophic lateral
sclerosis (ALS), as measured with the King's Clinical Staging System, and cognitive and …

Targeting NLRP3 inflammasome for neurodegenerative disorders

J Yao, Z Wang, W Song, Y Zhang - Molecular Psychiatry, 2023 - nature.com
Neuroinflammation is a key pathological feature in neurological diseases, including
Alzheimer's disease (AD). The nucleotide-binding domain leucine-rich repeat-containing …