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Human demyelinating disease and the polyomavirus JCV.
1Center for Neurovirology and Cancer Biology, College of Science and Technology, Temple University, Philadelphia, PA 19122, USA. kamel.khalili@temple.edu
Summary
Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease caused by the JC virus (JCV). Severe immunosuppression can allow JCV to replicate, increasing the risk of PML.
Area of Science:
- Neuroscience
- Virology
- Immunology
Background:
- Demyelinating diseases, including hereditary leukodystrophies, multiple sclerosis (MS), and progressive multifocal leukoencephalopathy (PML), affect the central and peripheral nervous systems.
- JC virus (JCV), a human neurotropic polyomavirus, is the causative agent of PML, a fatal demyelinating disease.
- JCV infections are typically latent due to immune system control, particularly cell-mediated immunity.
Purpose of the Study:
- To elucidate the role of JC virus (JCV) in the pathogenesis of progressive multifocal leukoencephalopathy (PML).
- To understand the factors contributing to the reactivation of latent JCV infections.
- To highlight the association between immunosuppression and the risk of developing PML.
Main Methods:
- Review of serological studies on JCV prevalence.
- Analysis of the characteristics of JCV, including its viral family and genome structure.
- Examination of the relationship between immune status and JCV replication.
Main Results:
- JCV is widespread in the human population but usually remains latent.
- Severe immunosuppression is a significant risk factor for JCV replication and the development of PML.
- PML primarily affects immunosuppressed individuals, particularly those with HIV/AIDS.
Conclusions:
- JC virus (JCV) is the etiological agent of progressive multifocal leukoencephalopathy (PML).
- Maintaining robust cell-mediated immunity is crucial for controlling JCV infections.
- Immunosuppressed states create a risk for JCV reactivation and subsequent PML development.