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Preparation and Immunostaining of the Myenteric Plexus of Prion-Infected Mice
Laura J Ellett1, Victoria A Lawson2
1Department of Pathology, The University of Melbourne, Medical Building, Parkville, VIC, 3010, Australia.
Abstract:
Intracerebral inoculation of mice with the M1000 strain of mouse-adapted human prions results in the consistent accumulation of PrPSc in the ileum of the gastrointestinal tract (GIT) of mice with clinical signs of prion disease. The accumulation of PrPSc in the ileum is accompanied by caspase activation and loss of immunoreactivity in subpopulations of neurons in the enteric nervous system. This suggests that like neurons in the central nervous system, cells in the enteric nervous system are also susceptible to prion-induced toxicity. In this chapter we describe the immunostaining of cells in myenteric plexus preparations of whole mounts prepared from the gastrointestinal tract of prion-infected mice.
Insights
Mouse-adapted human prions cause PrPSc accumulation in the ileum, leading to enteric nervous system neuron damage. This indicates gastrointestinal tract cells are vulnerable to prion toxicity.
Area of Science:
- Neuroscience
- Gastroenterology
- Prion Disease Research
Background:
- Prion diseases involve the accumulation of abnormal prion protein (PrPSc).
- Previous research focused on central nervous system (CNS) prion pathology.
- The gastrointestinal tract's (GIT) role in prion disease pathogenesis is less understood.
Purpose of the Study:
- To investigate prion protein (PrPSc) accumulation in the ileum of mice infected with a human prion strain.
- To examine the impact of PrPSc accumulation on the enteric nervous system (ENS).
- To determine if ENS cells are susceptible to prion-induced toxicity.
Main Methods:
- Intracerebral inoculation of mice with the M1000 strain of mouse-adapted human prions.
- Analysis of PrPSc accumulation in the ileum using immunostaining.
- Assessment of caspase activation and neuronal immunoreactivity in the ENS.
- Examination of myenteric plexus whole mount preparations from infected mice.
Main Results:
- Consistent PrPSc accumulation was observed in the ileum of prion-infected mice.
- PrPSc accumulation correlated with caspase activation in the ENS.
- Loss of immunoreactivity was noted in subpopulations of ENS neurons.
- These changes occurred alongside clinical signs of prion disease.
Conclusions:
- The enteric nervous system (ENS) is susceptible to prion-induced toxicity, similar to the central nervous system (CNS).
- PrPSc accumulation in the ileum contributes to ENS pathology during prion disease.
- These findings highlight the gastrointestinal tract as a potential site for prion pathogenesis.
