The spectrum of cognitive dysfunction in amyotrophic lateral sclerosis: an update

KA Jellinger - International Journal of Molecular Sciences, 2023 - mdpi.com
Cognitive dysfunction is an important non-motor symptom in amyotrophic lateral sclerosis
(ALS) that has a negative impact on survival and caregiver burden. It shows a wide spectrum …

Neural circuit and synaptic dysfunctions in ALS-FTD pathology

S Mora, I Allodi - Frontiers in Neural Circuits, 2023 - frontiersin.org
Action selection is a capital feature of cognition that guides behavior in processes that range
from motor patterns to executive functions. Here, the ongoing actions need to be monitored …

FUS alters circRNA metabolism in human motor neurons carrying the ALS-linked P525L mutation

A Colantoni, D Capauto, V Alfano, E D'Ambra… - International Journal of …, 2023 - mdpi.com
Deregulation of RNA metabolism has emerged as one of the key events leading to the
degeneration of motor neurons (MNs) in Amyotrophic Lateral Sclerosis (ALS) disease …

Patterns of synaptic loss in human amyotrophic lateral sclerosis spinal cord: a clinicopathological study

O Aousji, S Feldengut, S Antonucci, M Schön… - Acta Neuropathologica …, 2023 - Springer
Abstract Amyotrophic Lateral Sclerosis (ALS) is mainly characterized by the degeneration of
corticospinal neurons and spinal α-motoneurons; vulnerable cells display prominent pTDP …

Integrative proteomics highlight presynaptic alterations and c-Jun misactivation as convergent pathomechanisms in ALS

A Aly, ZI Laszlo, S Rajkumar, T Demir, N Hindley… - Acta …, 2023 - Springer
Abstract Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease mainly
affecting upper and lower motoneurons. Several functionally heterogeneous genes have …

Emerging perspectives of synaptic biomarkers in ALS and FTD

K Krishnamurthy, RK Pradhan - Frontiers in Molecular Neuroscience, 2024 - frontiersin.org
Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD) are debilitating
neurodegenerative diseases with shared pathological features like transactive response …

Emerging biophysical techniques for probing synaptic transmission in neurodegenerative disorders

MB Kale, NL Wankhede, AK Bishoyi, S Ballal, R Kalia… - Neuroscience, 2024 - Elsevier
Plethora of research has shed light on the critical role of synaptic dysfunction in various
neurodegenerative disorders (NDDs), including Alzheimer's disease (AD), Parkinson's …

Neuronal dysfunction caused by FUSR521G promotes ALS-associated phenotypes that are attenuated by NF-κB inhibition

MC Pelaez, A Desmeules, PA Gelon, B Glasson… - Acta Neuropathologica …, 2023 - Springer
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are related
neurodegenerative diseases that belong to a common disease spectrum based on …

Unraveling the Genetic Landscape of Neurological Disorders: Insights into Pathogenesis, Techniques for Variant Identification, and Therapeutic Approaches

Z Firdaus, X Li - International journal of molecular sciences, 2024 - mdpi.com
Genetic abnormalities play a crucial role in the development of neurodegenerative disorders
(NDDs). Genetic exploration has indeed contributed to unraveling the molecular …

Functional genomics and systems biology in human neuroscience

G Konopka, A Bhaduri - Nature, 2023 - nature.com
Neuroscience research has entered a phase of key discoveries in the realm of
neurogenomics owing to strong financial and intellectual support for resource building and …