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Related Concept Videos

Parkinson Disease ll: Pathophysiology01:24

Parkinson 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...
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Myasthenia Gravis ll: Pathophysiology01:22

Myasthenia Gravis ll: Pathophysiology

The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...
Parkinson's Disease: Overview01:15

Parkinson'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...
Multiple Sclerosis l: Introduction01:19

Multiple Sclerosis l: Introduction

Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
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Disorders of the Nervous Tissue

Nervous tissue is a vital component of the human body's communication system, enabling us to perceive and respond to stimuli. However, like all other tissues, it is vulnerable to disorders and diseases that can significantly impact our neurological functioning.
Homeostatic Imbalances:
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Updated: May 8, 2026

Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis
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Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis

Published on: July 16, 2021

Amyotrophic lateral sclerosis: cell vulnerability or system vulnerability?

Kevin Talbot1

  • 1Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.

Journal of Anatomy
|September 10, 2013
PubMed
Summary
This summary is machine-generated.

Amyotrophic lateral sclerosis (ALS) is a complex neurodegenerative disease involving genetic and environmental factors. Research explores cellular dysfunction and system-level degeneration to understand ALS initiation and propagation.

Keywords:
C9orf72TDP-43amyotrophic lateral sclerosismotor neuron disease

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ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
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ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
15:48

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies

Published on: July 29, 2007

Area of Science:

  • Neuroscience
  • Genetics
  • Pathology

Background:

  • Amyotrophic lateral sclerosis (ALS) is a complex neurodegenerative disease with overlaps with frontotemporal dementia (FTD).
  • ALS is now understood as a clinicopathological syndrome influenced by genetic susceptibility, cellular homeostasis loss, and environmental factors.
  • Recent genetic discoveries have spurred in vitro and in vivo models, revealing disruptions in biochemical pathways and cellular compartments.

Purpose of the Study:

  • To explore the complex interplay between cellular and systems neurobiology in ALS pathogenesis.
  • To investigate the initiation and propagation mechanisms of ALS at both cellular and system levels.
  • To bridge the gap between cellular degeneration and system-level degeneration for improved disease modeling and therapeutic development.

Main Methods:

  • Review of current research on ALS pathogenesis, including genetic associations and cellular pathway disruptions.
  • Analysis of clinical observations of focal onset and contiguous spread in ALS progression.
  • Integration of electrophysiological and brain-imaging studies to support the 'system degeneration' concept.

Main Results:

  • Evidence implicates pathways such as protein misfolding, mRNA splicing, oxidative stress, and mitochondrial dysfunction in ALS.
  • Clinical presentation suggests ALS progresses via specific anatomical pathways, supporting a 'system degeneration' model.
  • A dichotomy exists between cellular pathology and system-level degeneration, raising questions about disease initiation and spread.

Conclusions:

  • Understanding the interaction between cellular and system degeneration is crucial for early disease modeling.
  • Elucidating these interactions will improve the development of sensitive markers for disease progression and therapy response.
  • Further research is needed to understand the biological basis of ALS's clinical and pathological heterogeneity.