- Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease that affects motor neurons.
- Symptoms: increasing weakness in one limb, difficulty walking, clumsiness of the hands, light twitching, impaired speech, and difficulty swallowing, eventual paralysis.
- Scientists studying fruit flies have discovered how genetic mutations disrupt functions in neurons and lead to neurodegenerative diseases, such as ALS.
- The use of the fruit fly is still in early stages, but this model has potential to aid in the discovery of treatments or even a cure for those with ALS.
The little girls adored their father. One day, during an intense game of “monkey in the middle,” they noticed something was very different about the way Daddy played. He was slower and weaker in his arms; just tossing the ball seemed to take every bit of strength he had. Eventually, the doctor diagnosed their strong and active daddy with Amyotrophic Lateral Sclerosis (ALS), or Lou Gehrig’s Disease, which affects people between the ages of 40 and 75, and is more common in men than in women. (“Diseases and Conditions. Amyotrophic lateral sclerosis,” 2014).ALS is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord (“What is ALS?,” 2010). When motor neurons die, the ability of the brain to initiate and control muscle movement is lost, which explained Daddy’s weakness in his game of “monkey in the middle.” Though the true cause of ALS is still unclear, there are identifiable symptoms of the disease. In the early stages, the symptoms of ALS include: increasing weakness in one limb, difficulty walking, clumsiness of the hands, light twitching, impaired speech, and difficulty swallowing – all symptoms Mommy had been noticing in Daddy. As the disease progresses, the family will watch as his weakness increases until eventually he becomes paralyzed (“Understanding ALS,” 2014).
The tiny Drosophila melanogaster, more commonly known as the fruit fly, could hold the answers to many of the mysteries surrounding ALS (“ALS Facts and Statistics,” 2014). Scientists are currently studying genes that are believed to play a role in the disease in hopes that they can develop targeted gene therapies. This research is being performed on fruit flies, an organism that has proven to be valuable in finding genes and molecular pathways integral in several neurological diseases (“ALS Facts and Statistics”, 2013). One study in fruit flies revealed a method of reducing the toxicity associated with a key ALS protein, a strategy to slow the loss of neuron function. Also studying the fruit fly, scientists at Johns Hopkins have discovered new ways that genetic mutations can disrupt functions in neurons and lead to neurodegenerative diseases, such as ALS, (Johns Hopkins Medicine, 2013). According to Nancy Bonini, a biology professor in the School of Arts & Sciences, “These model systems are very fast and simpler than mammalian models. They allow us to focus on conserved pathways, and can be remarkably powerful for giving us insight into pathways involved in disease” (“ALS Facts and Statistics”, 2013).
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