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Related Concept Videos

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...
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 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...
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.
Alterations in Muscle Tone lll01:11

Alterations in Muscle Tone lll

Rigidity and myotonia are distinct abnormalities of muscle tone that affect resistance and relaxation during movement. Although both involve altered muscle contraction, they arise from different neurological and muscular mechanisms.CharacteristicsRigidity is characterized by uniform resistance to passive movement across the entire range, independent of speed, affecting flexors and extensors equally. It may appear as lead-pipe rigidity (smooth, constant resistance) or cogwheel rigidity...
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 7, 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: Problems and prospects.

Jemeen Sreedharan1, Robert H Brown

  • 1Babraham Institute, Cambridge, United Kingdom; Department of Neurology, University of Massachusetts Medical School, Worcester, MA; Department of Neurobiology, University of Massachusetts Medical School, Worcester, MA.

Annals of Neurology
|September 17, 2013
PubMed
Summary

Amyotrophic lateral sclerosis (ALS) research reveals protein instability and RNA processing issues are key to motor neuron death. New technologies promise innovative therapies for this neurodegenerative disease.

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Intraspinal Cell Transplantation for Targeting Cervical Ventral Horn in Amyotrophic Lateral Sclerosis and Traumatic Spinal Cord Injury
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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

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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

Intraspinal Cell Transplantation for Targeting Cervical Ventral Horn in Amyotrophic Lateral Sclerosis and Traumatic Spinal Cord Injury
10:49

Intraspinal Cell Transplantation for Targeting Cervical Ventral Horn in Amyotrophic Lateral Sclerosis and Traumatic Spinal Cord Injury

Published on: September 18, 2011

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
  • Molecular Biology

Background:

  • Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease, often co-occurring with frontotemporal dementia.
  • Genetics of inherited ALS cases illuminate disease mechanisms, focusing on protein instability and RNA processing.
  • Non-neuronal cells and distal motor neuron components are increasingly recognized as participants in ALS pathology.

Purpose of the Study:

  • To review current understanding of ALS pathobiology based on genetic insights.
  • To highlight emerging research directions and technological advancements in ALS research.
  • To underscore the need for new therapeutic strategies and biomarkers for ALS.

Main Methods:

  • Analysis of genetic studies, particularly focusing on SOD1, TDP43, FUS, and C9orf72 genes.
  • Review of research implicating non-neuronal cells and axonal/terminal events in ALS.
  • Examination of current therapeutic approaches and clinical trials, including stem cell therapies.

Main Results:

  • Protein conformational instability (e.g., SOD1) and RNA processing defects (e.g., TDP43, FUS, C9orf72) are central to ALS.
  • Neuroinflammation and distal motor neuron pathology contribute significantly to disease progression.
  • Limited therapeutic options exist, with riluzole being the only FDA-approved drug; several therapies are in clinical trials.

Conclusions:

  • High-throughput sequencing and advanced technologies are crucial for identifying new ALS genes and pathways.
  • Development of robust biomarkers and validation infrastructures is essential for accelerating clinical trials.
  • Future research focusing on genetics, epigenetics, environmental factors, and novel therapeutic modalities offers hope for effective ALS treatments.