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The Case of the Malfunctioning Neuron

When Cellular Processes Go Wrong


Kirsten L. Hokeness
Science and Technology Department
Bryant University


This flipped case study tells the story of Joyce, a biology student who notices the development of some unusual symptoms (foot slapping and slurred speech) in her mother. In an effort to understand the cause, Joyce views a documentary-style trigger video (created by the case author) that suggests to Joyce that her mom may in fact have amyotrophic lateral sclerosis or ALS. The rest of the case walks Joyce through understanding how normal neurons compare to neurons in ALS patients and how that might affect muscle function. The case explores the link between genes, particularly SOD-1, to the formation of malformed proteins and its potential role in the development of ALS. The case concludes with a discussion of drug development and highlights the timeline and costs associated with drug discovery as Joyce becomes concerned about the lack of drugs in the pipeline for ALS, which her mother is ultimately diagnosed with. The case is appropriate for a number of classes including general biology, biotechnology, anatomy and physiology, upper level-cell biology, or any human health and disease-related course.


  • Understand and explain the basic structure and function of neurons.
  • Explain what can happen when motor neuron function goes awry using ALS as a model neurodegenerative disease.
  • Investigate and explain the possible mechanisms for the development of ALS.
  • Connect the biological concept of proper protein folding with the possible development of neurological disease.
  • Explain the process of drug development.


neurons; transcription; translation; genes; mutations; ALS; amyotrophic lateral sclerosis; Lou Gehrig's disease; Lou Gehrig; drug development; drug discovery; neuron function

Topical Areas

Regulatory issues

Educational Level

Undergraduate lower division



Type / Methods

Flipped, Interrupted



Subject Headings

Biology (General)  |   Cell Biology  |   Molecular Biology  |   Physiology  |   Genetics / Heredity  |   Neuroscience  |   Science (General)  |  

Date Posted


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The following video(s) are recommended for use in association with this case study.

  • 2-Minute Neuroscience: The Neuron
    This video gives a brief overview of the parts of a neuron and how it functions in signaling. Running time: 1:47 min. Created by Neuroscientifically Challenged, 2014.
  • A Personal Story of ALS: Told by Kirsten Hokeness
    This video narrates one woman’s journey as she learns that her mother is diagnosed with amyotrophic lateral sclerosis (ALS), for which there is no cure. The signs and symptoms of the disease are described here as she talks about her mother’s progression with the deadly illness and highlights some of the emotional turmoil the family is left to face. Running time: 5:01 min. Created by Kirsten L. Hokeness for the National Center for Case Study Teaching in Science, 2017.
  • 2-Minute Neuroscience: Amyotrophic Lateral Sclerosis (ALS)
    This brief video gives an overview of ALS symptoms and pathophysiology. Running time: 1:58 min. Created by Neuroscientifically Challenged, 2017.
  • Protein Synthesis
    This animation gives an overview of protein synthesis, transcription, and translation. Running time: 4:47 min. Created by Wisc-Online, 2017.