Related Experiment Video
Updated: Mar 23, 2026

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
Published on: July 29, 2007
Quo vadis motor neuron disease?
Rubika Balendra1, Rickie Patani1
1Rubika Balendra, Department of Neurodegenerative Disease, Institute of Neurology, University College London, London WC1N 3BG, United Kingdom.
Abstract:
Motor neuron disease (MND), also known as amyotrophic lateral sclerosis, is a relentlessly progressive neurodegenerative condition that is invariably fatal, usually within 3 to 5 years of diagnosis. The aetio-pathogenesis of MND remains unresolved and no effective treatments exist. The only Food and Drug Administration approved disease modifying therapy is riluzole, a glutamate antagonist, which prolongs survival by up to 3 mo. Current management is largely symptomatic/supportive. There is therefore a desperate and unmet clinical need for discovery of disease mechanisms to guide novel therapeutic strategy. In this review, we start by introducing the organizational anatomy of the motor system, before providing a clinical overview of its dysfunction specifically in MND. We then summarize insights gained from pathological, genetic and animal models and conclude by speculating on optimal strategies to drive the step change in discovery, which is so desperately needed in this arena.
Insights
Motor neuron disease (MND), or amyotrophic lateral sclerosis, is a fatal neurodegenerative condition with no cure. This review explores MND mechanisms and discusses strategies for developing effective treatments.
Area of Science:
- Neurodegenerative diseases
- Neurobiology
- Neurology
Background:
- Motor neuron disease (MND), also known as amyotrophic lateral sclerosis (ALS), is a progressive, fatal neurodegenerative disorder.
- Current understanding of MND's etiology and pathogenesis is incomplete, with no definitive cure available.
- Riluzole, a glutamate antagonist, is the sole FDA-approved therapy, offering modest survival benefits.
Purpose of the Study:
- To provide a comprehensive overview of the motor system's anatomy and its dysfunction in MND.
- To summarize current knowledge on MND pathogenesis derived from pathological, genetic, and animal studies.
- To propose strategies for accelerating the discovery of novel therapeutic targets for MND.
Main Methods:
- Review of existing literature on motor system anatomy and MND.
- Analysis of pathological findings in MND.
- Examination of genetic factors implicated in MND.
- Evaluation of insights from animal models of MND.
Main Results:
- The review details the structural and functional organization of the motor system.
- It highlights key clinical features and progression patterns of MND.
- Insights from diverse research avenues underscore the complexity of MND.
Conclusions:
- There is a critical unmet need for effective treatments for MND.
- Understanding disease mechanisms is paramount for developing novel therapeutic strategies.
- A multi-faceted approach combining pathological, genetic, and model system insights is crucial for advancing MND research.
More Related Videos
06:35In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration
Published on: June 15, 2018
06:12Dissection of the Transversus Abdominis Muscle for Whole-mount Neuromuscular Junction Analysis
Published on: January 11, 2014
Related Concept Videos
Parkinson's Disease: Overview
Myasthenia Gravis: Overview and Treatment
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
The Neuromuscular Junction
Neural Regulation
Myasthenia Gravis: Diagnostic Tests
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
Tumor Progression
Colon cancer is one of the best-documented examples of tumor progression. Early mutation in the APC gene in colon cells causes a small growth on the colon wall called a polyp. With time, this polyp grows into a benign, pre-cancerous tumor. Further...