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Consider a small protein molecule in your brain that has the ability to change shape and, as a result, can trigger a chain reaction that leads to a damaging neurogentarive disease. Unfortunately, this protein exists and is known as a prion, which are misfolded proteins that can cause other healthy proteins to change. In this article, we will look at the role of prions and their relationship to the neurological disease Alzheimer's.
What Are Prions and Why Are They Important?
Your body depends on proteins to perform everything from helping with cell functions to body functions. Prions are proteins that are generally found in the brain that are misfolded that can cause normal proteins in the brain to fold abnormally (Zhou, 2013). Prion, which stands for proteinaceous infectious particle and normally appears as a coil, is a protein that is present in everyone but affects different people due to their different structures. Prions come in two varieties: PrPC and PrPSc. Despite their similarity in sound, one is very harmful to brain function (Shetty, 2016).
PrPC: These proteins are present on cell membranes. They are thought to be crucial for cell adhesion and intracellular signaling. However, because the problems pertaining to its function remain unresolved, research is still being conducted.
PrPSc is a protease-resistant prion that causes disease. By altering its shape, it modifies the PrPC. Its interactions and connections with other proteins are altered by the structural change!
Since prions lack DNA, they cannot survive on their own and are not contagious. Moreover, prions are resistant to protease (an enzyme) degradation, despite the fact that the majority of proteins in cells are easily broken down. This is one of the reasons these "rogue" disease agents can spread so relentlessly (Zhou, 2013). A prion's interaction with a normal protein sets off a series of events that result in the misfolding of other proteins. This buildup of misfolded proteins causes serious brain damage and cell death.
The Link Between Prions and Alzheimer’s Disease
Recent studies on mice have attempted to determine the precise function of prion, but the results have shown that mice do not have prions because they do not have the gene that gives the body instructions to make them (Britannica Kids, 2025), demonstrating that because mice lack prions, they are immune to a variety of neurogenerative diseases.
According to some research, prions are the primary cause of Alzheimer's disease (AD), a progressive neurogenerative illness that causes memory loss, brain atrophy, and cognitive decline in its victims.
The Role of Protein Misfolding in Brain Damage
Protein misfolding in Alzheimer's disease occurs as a cascade effect. Amyloid-beta (Aβ), a sticky starchy protein found on the brain (Kennedy, 2015), clumps together and forms plaques, disrupting cell communication. In response, tau proteins, which help with structure inside neurons, begin to misfold, resulting in neuronal tangles. These tangles disrupt the neuron's internal transport system, preventing necessary nutrients from entering the cell.
As more proteins misfold and accumulate, neurons become dysfunctional and ultimately die. This widespread neuronal loss causes the hallmark symptoms of Alzheimer's disease, such as memory impairment, confusion, and difficulty performing daily tasks.
How Prions and AD Affect Brain Communication
Prions also disrupt cell-to-cell communication. They do not use phones to communicate like us; instead, they use molecules known as signaling molecules, such as hormones or neurotransmitters. They are transmitted in the brain via a synapse, which connects two neurons. Prions can damage the synapse, impairing the release of signaling molecules and thus preventing brain cells from communicating with one another (Shetty, 2016).
Additionally, a prion-like mechanism can cause inflammation in the brain. Microglial cells, which are responsible for cleaning up cellular debris, become overactive when interacting with prions, resulting in excessive inflammation and additional neuronal damage.
Understanding the prion-like behavior of proteins in Alzheimer's disease is critical for developing new treatments. If scientists can find ways to prevent or halt misfolding, they may be able to slow or even stop the progression of Alzheimer's disease. Research into prion-based therapies could lead to future breakthroughs in neurodegenerative disease treatment, and is a fascinating field!
Reference list
Britannica Kids. (2025). Prion. [online] Available at: https://kids.britannica.com/students/article/Prion/341242 [Accessed 30 Jan. 2025].
BYJUS. (n.d.). What Are Prions? - Structure, And Diseases Caused By Prions. [online] Available at: https://byjus.com/biology/prions/.
Kennedy, K.M. (2015). Amyloid Beta - an overview | ScienceDirect Topics. [online] www.sciencedirect.com. Available at: https://www.sciencedirect.com/topics/medicine-and-dentistry/amyloid-beta.
Nature.com. (2024). Prions - Latest research and news | Nature. [online] Available at: https://www.nature.com/subjects/prions.
Shetty, V. (2016). Prions: Definition, Structure, Function, Replication and Diseases. [online] Science ABC. Available at: https://www.scienceabc.com/pure-sciences/what-are-prions.html.
Zhou (2013). Alzheimer’s Disease and Prion Protein. Intractable & Rare Diseases Research. doi:https://doi.org/10.5582/irdr.2013.v2.2.35.
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