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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...
Motor Units00:46

Motor Units

A motor unit consists of two main components: a single efferent motor neuron (i.e., a neuron that carries impulses away from the central nervous system) and all of the muscle fibers it innervates. The motor neuron may innervate multiple muscle fibers, which are single cells, but only one motor neuron innervates a single muscle fiber.
Motor Units01:13

Motor Units

The motor unit is a fundamental component of the neuromuscular system and plays a crucial role in coordinating muscle contractions. It consists of a somatic motor neuron, which connects and controls multiple skeletal muscle fibers, forming a single functional segment. The axon of the motor neuron branches out and establishes synaptic connections known as neuromuscular junctions with individual muscle fibers within the motor unit.
Motor units come in different sizes, with smaller units...
Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
Alterations in Muscle Tone ll01:12

Alterations in Muscle Tone ll

Alterations in muscle tone are common manifestations of neurological disorders and reflect dysfunction within different nervous system regions. Spasticity, paratonia, and dystonia represent distinct forms of hypertonia, each with unique mechanisms, clinical features, and diagnostic importance.CharacteristicsSpasticity happens from upper motor neuron lesions and is characterized by velocity-dependent resistance to passive movement. Clinical features include:Exaggerated deep tendon reflexesClonus...
Chemical Synapses01:26

Chemical Synapses

Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...

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Related Experiment Video

Updated: Jun 1, 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

[Motor neuron diseases].

S Petri1, T Meyer

  • 1Klinik für Neurologie-OE 7210, Medizinische Hochschule Hannover, Hannover. Petri.Susanne@mh-hannover.de

Der Nervenarzt
|June 1, 2011
PubMed
Summary
This summary is machine-generated.

Motor neuron diseases (MND), including amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA), share common RNA regulation disturbances. Understanding these molecular pathways is key to developing targeted treatments for motor neuron degeneration.

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Last Updated: Jun 1, 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

Generation of Human Motor Units with Functional Neuromuscular Junctions in Microfluidic Devices
10:48

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In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration
06:35

In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration

Published on: June 15, 2018

Area of Science:

  • Neuroscience
  • Genetics
  • Molecular Biology

Context:

  • Motor neuron diseases (MND) encompass a diverse group of neurodegenerative disorders.
  • Key examples include amyotrophic lateral sclerosis (ALS), proximal spinal muscular atrophy (SMA), and hereditary motor neuropathies (HMN).
  • Both familial and sporadic forms of these conditions are recognized.

Purpose:

  • To explore the molecular underpinnings of motor neuron degeneration in MND.
  • To identify common pathogenic mechanisms across different MND subtypes.
  • To establish a foundation for developing targeted molecular therapies.

Summary:

  • Intraneuronal protein inclusions, involving TDP-43, FUS, SOD1, or ataxin-2, are a hallmark of ALS.
  • These proteins are multifunctional DNA/RNA-binding proteins crucial for transcription regulation.
  • SMA and HMN involve different genes, but their products may also play roles in RNA processing.
  • Dysregulation of RNA processing is a potential shared pathophysiological feature in MND.

Impact:

  • Elucidating common pathways in motor neuron degeneration is crucial.
  • This understanding facilitates the development of molecularly targeted treatment strategies.
  • Advances benefit both ALS and other hereditary/sporadic lower motor neuron syndromes.