Synaptic dysfunction in ALS and FTD: anatomical and molecular changes provide insights into mechanisms of disease
PA Gelon, PA Dutchak, CF Sephton - Frontiers in Molecular …, 2022 - frontiersin.org
Synaptic loss is a pathological feature of all neurodegenerative diseases including
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). ALS is a disease of …
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). ALS is a disease of …
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 …
affecting upper and lower motoneurons. Several functionally heterogeneous genes have …
Synaptopathy: presynaptic convergence in frontotemporal dementia and amyotrophic lateral sclerosis
EL Clayton, L Huggon, MA Cousin, S Mizielinska - Brain, 2024 - academic.oup.com
Frontotemporal dementia and amyotrophic lateral sclerosis are common forms of
neurodegenerative disease which share overlapping genetics and pathologies. Crucially …
neurodegenerative disease which share overlapping genetics and pathologies. Crucially …
ALS-associated C21ORF2 variant disrupts DNA damage repair, mitochondrial metabolism, neuronal excitability and NEK1 levels in human motor neurons
P Zelina, AA de Ruiter, C Kolsteeg… - Acta neuropathologica …, 2024 - Springer
Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease leading to
motor neuron loss. Currently mutations in> 40 genes have been linked to ALS, but the …
motor neuron loss. Currently mutations in> 40 genes have been linked to ALS, but the …
Neural Differentiation and spinal cord organoid generation from induced pluripotent stem cells (iPSCs) for ALS modelling and inflammatory screening
R Guo, Y Chen, J Zhang, Z Zhou, B Feng, X Du… - Molecular …, 2024 - Springer
C9orf72 genetic mutation is the most common genetic cause of ALS/FTD accompanied by
abnormal protein insufficiency. Induced pluripotent stem cell (iPSC)-derived two …
abnormal protein insufficiency. Induced pluripotent stem cell (iPSC)-derived two …
Als patient-derived motor neuron networks exhibit microscale dysfunction and mesoscale compensation rendering them highly vulnerable to perturbation
V Fiskum, N Winter-Hjelm, N Christiansen, A Sandvig… - Biorxiv, 2024 - biorxiv.org
Amyotrophic lateral sclerosis affects upper and lower motor neurons, causing progressive
neuropathology leading to structural and functional alterations of affected neural networks …
neuropathology leading to structural and functional alterations of affected neural networks …
Cellular and axonal transport phenotypes due to the C9ORF72 HRE in iPSC motor and sensory neurons
Induced pluripotent stem cell (iPSC)-derived motor neurons (MNs) from patients with
amyotrophic lateral sclerosis (ALS) and the C9ORF72 hexanucleotide repeat expansion …
amyotrophic lateral sclerosis (ALS) and the C9ORF72 hexanucleotide repeat expansion …
TDP-43 regulates LC3ylation in neural tissue through ATG4B cryptic splicing inhibition
P Torres, S Rico-Rios, M Ceron-Codorniu… - Acta …, 2024 - Springer
Amyotrophic lateral sclerosis (ALS) is an adult-onset motor neuron disease with a mean
survival time of three years. The 97% of the cases have TDP-43 nuclear depletion and …
survival time of three years. The 97% of the cases have TDP-43 nuclear depletion and …
[HTML][HTML] Fast and efficient synaptosome isolation and post-synaptic density enrichment from hiPSC-motor neurons by biochemical sub-cellular fractionation
S Rajkumar, TM Böckers, A Catanese - STAR protocols, 2023 - Elsevier
We describe here a time-efficient, in-house protocol for synaptosome isolation and
enrichment of the post-synaptic density (PSD) from hiPSC-derived motor neurons. By using …
enrichment of the post-synaptic density (PSD) from hiPSC-derived motor neurons. By using …
Neuronal models of TDP-43 proteinopathy display reduced axonal translation, increased oxidative stress, and defective exocytosis
A Pisciottani, L Croci, F Lauria, C Marullo… - Frontiers in Cellular …, 2023 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a progressive, lethal neurodegenerative disease
mostly affecting people around 50–60 years of age. TDP-43, an RNA-binding protein …
mostly affecting people around 50–60 years of age. TDP-43, an RNA-binding protein …