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Related Concept Videos

Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

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The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
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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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Chemical Synapses

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Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
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The nervous system consists of complex motor neuron circuits, including upper motor neurons originating from the cerebral cortex and lower motor neurons starting in the spinal cord, coordinating both voluntary and involuntary movements. Among these, somatic motor neurons activate skeletal muscles and are classified into alpha, beta, and gamma types. Alpha neurons are vital for voluntary movement coordination, while gamma neurons adjust muscle spindle sensitivity, and the function of beta...
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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...
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Digestion begins with a cephalic phase that prepares the digestive system to receive food. When our brain processes visual or olfactory information about food, it triggers impulses in the cranial nerves innervating the salivary glands and stomach to prepare for food.
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Related Experiment Video

Updated: Aug 29, 2025

Electrophysiological Motor Unit Number Estimation MUNE Measuring Compound Muscle Action Potential CMAP in Mouse Hindlimb Muscles
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Motoneuron Diseases.

Francesco Lotti1, Serge Przedborski2

  • 1Departments of Neurology, Pathology & Cell Biology, and Neuroscience, College of Physicians and Surgeons, Columbia University, New York, NY, USA.

Advances in Neurobiology
|September 6, 2022
PubMed
Summary
This summary is machine-generated.

Motoneuron diseases (MNDs) are progressive paralytic disorders caused by nerve cell loss. Their varied presentation suggests complex genetic and environmental factors influence disease progression.

Keywords:
Amyotrophic lateral sclerosisC9ORF72Cell autonomyFUSMotoneuron diseasesMotor cortexNeurodegenerationSpinal cordSpinal muscular atrophySuperoxide dismutase-1TDP43

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Modeling Charcot-Marie-Tooth Disease In Vitro by Transfecting Mouse Primary Motoneurons
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Last Updated: Aug 29, 2025

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In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration
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Modeling Charcot-Marie-Tooth Disease In Vitro by Transfecting Mouse Primary Motoneurons

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Area of Science:

  • Neurology
  • Neuroscience
  • Genetics

Background:

  • Motoneuron diseases (MNDs) encompass a range of progressive paralytic conditions.
  • These disorders are defined by the degeneration of upper and/or lower motoneurons.
  • MNDs manifest across a spectrum from birth to adulthood with diverse clinical presentations.

Purpose of the Study:

  • To explore the heterogeneity and underlying mechanisms of motoneuron diseases.
  • To investigate the influence of genetic and environmental factors on MND variability.
  • To understand the multifactorial nature of motoneuron degeneration.

Main Methods:

  • Review of existing literature on motoneuron diseases.
  • Analysis of clinical presentations and genetic data.
  • Examination of cellular mechanisms in motoneuron degeneration.

Main Results:

  • MNDs are characterized by the loss of corticospinal and/or spinal motoneurons.
  • Clinical presentation varies significantly, even in genetically defined forms.
  • Evidence suggests both cell-autonomous and non-cell-autonomous mechanisms contribute to pathology.

Conclusions:

  • Motoneuron diseases are complex and heterogeneous disorders.
  • Genetic and environmental factors likely modify disease course and presentation.
  • Multifactorial pathogenic processes involving various cellular mechanisms underlie motoneuron degeneration.