Motor neuron susceptibility in ALS/FTD

AMG Ragagnin, S Shadfar, M Vidal… - Frontiers in …, 2019 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
death of both upper and lower motor neurons (MNs) in the brain, brainstem and spinal cord …

Oxidative stress as a potential mechanism underlying membrane hyperexcitability in neurodegenerative diseases

R Pardillo-Díaz, P Pérez-García, C Castro… - Antioxidants, 2022 - mdpi.com
Neurodegenerative diseases are characterized by gradually progressive, selective loss of
anatomically or physiologically related neuronal systems that produce brain damage from …

Synaptic FUS accumulation triggers early misregulation of synaptic RNAs in a mouse model of ALS

S Sahadevan, KM Hembach, E Tantardini… - Nature …, 2021 - nature.com
Mutations disrupting the nuclear localization of the RNA-binding protein FUS characterize a
subset of amyotrophic lateral sclerosis patients (ALS-FUS). FUS regulates nuclear RNAs …

Synaptic proteomics reveal distinct molecular signatures of cognitive change and C9ORF72 repeat expansion in the human ALS cortex

ZI Laszlo, N Hindley, A Sanchez Avila, RA Kline… - Acta neuropathologica …, 2022 - Springer
Increasing evidence suggests synaptic dysfunction is a central and possibly triggering factor
in Amyotrophic Lateral Sclerosis (ALS). Despite this, we still know very little about the …

[HTML][HTML] Single-cell RNA-seq analysis of the brainstem of mutant SOD1 mice reveals perturbed cell types and pathways of amyotrophic lateral sclerosis

W Liu, S Venugopal, S Majid, IS Ahn, G Diamante… - Neurobiology of …, 2020 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease in which motor neurons
throughout the brain and spinal cord progressively degenerate resulting in muscle atrophy …

[HTML][HTML] A critical evaluation of current concepts in cerebral palsy

JE Brandenburg, MJ Fogarty, GC Sieck - Physiology, 2019 - journals.physiology.org
Spastic cerebral palsy (CP), despite the name, is not consistently identifiable by specific
brain lesions. CP animal models focus on risk factors for development of CP, yet few …

Glutamate transport and preterm brain injury

S Pregnolato, E Chakkarapani, AR Isles… - Frontiers in …, 2019 - frontiersin.org
Preterm birth complications are the leading cause of child death worldwide and a top global
health priority. Among the survivors, the risk of life-long disabilities is high, including cerebral …

[HTML][HTML] Evolution and functional differentiation of the diaphragm muscle of mammals

MJ Fogarty, GC Sieck - Comprehensive Physiology, 2019 - ncbi.nlm.nih.gov
Symmorphosis is a concept of economy of biological design, whereby structural properties
are matched to functional demands. According to symmorphosis, biological structures are …

[HTML][HTML] Structure and function of the mammalian neuromuscular junction

LA Davis, MJ Fogarty, A Brown… - Comprehensive …, 2022 - ncbi.nlm.nih.gov
The mammalian neuromuscular junction (NMJ) comprises a presynaptic terminal, a
postsynaptic receptor region on the muscle fiber (endplate), and the perisynaptic (terminal) …

Amyotrophic lateral sclerosis as a synaptopathy

MJ Fogarty - Neural regeneration research, 2019 - journals.lww.com
The synapse is an incredibly specialized structure that allows for the coordinated
communication of information from one neuron to another. When assembled into circuits …