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Related Concept Videos

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...
Cross-bridge Cycle01:26

Cross-bridge Cycle

As muscle contracts, the overlap between the thin and thick filaments increases, decreasing the length of the sarcomere—the contractile unit of the muscle—using energy in the form of ATP. At the molecular level, this is a cyclic, multistep process that involves binding and hydrolysis of ATP, and movement of actin by myosin.
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...
Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
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 leads...
Parkinson Disease l: Introduction01:24

Parkinson Disease l: Introduction

Parkinson’s disease is a chronic, progressive neurodegenerative disorder that primarily affects movement. It is characterized by motor symptoms such as resting tremors, muscle rigidity, bradykinesia (slowness of movement), and postural instability. Patients may notice hand tremors at rest, stiffness during movement, or a shuffling gait. In addition to motor features, non-motor symptoms include sleep disturbances, mood and behavioral changes, constipation, and cognitive impairment, all of which...

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Related Experiment Video

Updated: May 13, 2026

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

Amyotrophic Lateral Sclerosis: A Review.

John Ravits1, Dominic Ferrey1, Betul Gundogdu1

  • 1ALS Clinical and Translational Research Programs, Department of Neurosciences, School of Medicine, University of California, San Diego, La Jolla.

JAMA
|May 11, 2026
PubMed
Summary
This summary is machine-generated.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease with no cure. Current FDA-approved treatments modestly slow progression, and multidisciplinary care improves quality of life for ALS patients.

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Last Updated: May 13, 2026

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

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Published on: July 29, 2007

Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
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Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis

Published on: March 4, 2014

Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis
08:59

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

Published on: July 16, 2021

Area of Science:

  • Neuroscience
  • Genetics
  • Pharmacology

Background:

  • Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease affecting approximately 25,000 individuals in the U.S.
  • Characterized by progressive muscle weakness due to motor neuron degeneration, ALS impacts speech, swallowing, and mobility, ultimately leading to respiratory failure.
  • While 85% of cases are sporadic, 15% are familial, with genetic factors like C9orf72 and SOD1 variants playing a significant role.

Purpose of the Study:

  • To provide an overview of Amyotrophic Lateral Sclerosis (ALS), including its clinical manifestations, genetic associations, and current therapeutic landscape.
  • To highlight the limitations in current ALS treatment and the impact of available therapies and supportive care.

Main Methods:

  • Review of clinical features and diagnostic criteria for ALS.
  • Analysis of genetic factors associated with familial ALS, including C9orf72 and SOD1.
  • Summary of FDA-approved disease-modifying therapies and their efficacy.

Main Results:

  • ALS diagnosis relies on clinical presentation, supported by electromyography.
  • Over 60 genes are linked to ALS, with C9orf72 and SOD1 being prominent in familial cases.
  • Riluzole and edaravone offer modest progression delay (2-4 months); Tofersen targets SOD1 variants.

Conclusions:

  • No curative therapies currently exist for ALS.
  • FDA-approved treatments provide modest benefits in slowing disease progression.
  • Multidisciplinary care teams significantly improve survival (4-7 months) and quality of life for ALS patients.