Related Experiment Video
Updated: May 24, 2026
08:59
Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis
Published on: July 16, 2021
Does interneuronal dysfunction contribute to neurodegeneration in amyotrophic lateral sclerosis?
Martin R Turner1, Matthew C Kiernan
1Oxford University Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, UK. martin.turner@clneuro.ox.ac.uk
Summary
Amyotrophic lateral sclerosis (ALS) involves motor system failure due to loss of central nervous system inhibition. Supporting interneuron function may offer a novel therapeutic strategy for this neurodegenerative disorder.
Area of Science:
- Neuroscience
- Neurology
Background:
- Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder causing motor system failure.
- Evidence suggests a loss of central nervous system inhibitory neuronal influence contributes to ALS pathogenesis.
- This points to an 'interneuronopathy' potentially explaining variable pathology in motor neuron populations.
Purpose of the Study:
- To explore the role of interneuron dysfunction in Amyotrophic Lateral Sclerosis (ALS).
- To investigate the potential of targeting interneuron function as a therapeutic strategy for ALS.
Main Methods:
- Review of clinical, histological, genetic, neurophysiological, neuroimaging, and neuropsychological studies.
- Analysis of evidence supporting the 'interneuronopathy' hypothesis in ALS.
- Theoretical modeling of excitotoxic pathways in ALS.
Main Results:
- A loss of central nervous system inhibitory function is implicated in ALS.
- Differences in cortical and spinal inhibitory networks may explain variable pathology.
- Unopposed glutamatergic activity could lead to an excitotoxic final common pathway.
Conclusions:
- Interneuron dysfunction is a key factor in Amyotrophic Lateral Sclerosis (ALS) pathogenesis.
- Targeting interneuron support presents a novel therapeutic avenue for ALS.
- Understanding interneuronopathy is crucial for developing effective ALS treatments.
Related Concept Videos
01:24Parkinson Disease ll: Pathophysiology
Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...
01:23Alzheimer Disease ll: Pathophysiology
Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and microglia. Abnormal...
01:37Neural Regulation
Digestion begins with a cephalic phase that prepares the digestive system to receive food. When our brain processes visual or olfactory information about food, it triggers impulses in the cranial nerves innervating the salivary glands and stomach to prepare for food.
01:15Parkinson's Disease: Overview
Neurodegenerative disorders are progressive diseases that cause irreversible damage and loss to neurons in specific brain areas. Examples of these disorders include Parkinson's disease, Alzheimer's disease, Multiple Sclerosis (MS), and Amyotrophic Lateral Sclerosis (ALS). These disorders share characteristics such as proteinopathies, selective neuronal vulnerability, and a complex interplay between genetic and environmental factors. The primary therapeutic goal for these conditions is to...
01:15Neurogenesis and Regeneration of Nervous Tissue
In the CNS, neurogenesis, the birth of new neurons from stem cells, is limited to the hippocampus in adults. In other regions of the brain and spinal cord, neurogenesis is almost non-existent due to inhibitory influences from neuroglia, especially oligodendrocytes, and the absence of growth-stimulating cues. The myelin produced by oligodendrocytes in the CNS inhibits neuronal regeneration. Furthermore, astrocytes proliferate rapidly after neuronal damage, forming scar tissue that physically...
01:29Alzheimer Disease l: Introduction
Alzheimer disease is a chronic, progressive, and irreversible neurodegenerative disorder and the most common cause of dementia in older adults. It leads to gradual neuronal loss, causing cognitive decline, behavioral changes, and loss of functional independence.Risk Factors and EtiologyThe disease is multifactorial. Age is the strongest risk factor, with prevalence doubling every 5 years after age 65. Genetic factors include mutations in genes such as APP, PSEN1, and PSEN2, which are associated...