Human demyelinating disease and the polyomavirus JCV.
Many human neurological diseases involve demyelination of the central and/or peripheral nervous systems. These include the hereditary leukodystrophies--which have a genetic basis; multiple sclerosis (MS)--where the underlying cause of demyelination remains unknown; and progressive multifocal leukoencephalopathy (PML)--where the etiology is well-established as being viral. The human neurotropic polyomavirus--JC virus (JCV)--is the etiologic agent of PML, a fatal demyelinating disease of the central nervous system that occurs mainly in immunosuppressed patients, especially those with HIV/AIDS. JCV belongs to the polyomavirus family of tumor viruses that are characterized by non-enveloped icosahedral capsids containing small, circular, double-stranded DNA genomes. Serological studies have shown that JCV is widespread throughout the human population, but infections are usually restricted by the immune system, particularly cell-mediated immunity, causing the virus to enter a latent phase. An important corollary of this is that situations of severe immunosuppression may permit JCV to replicate and are thus a risk factor for PML.
Khalili
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K
Center for Neurovirology and Cancer Biology, College of Science and Technology, Temple University, Philadelphia, PA 19122, USA. kamel.khalili@temple.edu
White
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MK
D003711:Demyelinating Diseases / Q000821:virology*; D006801:Humans; D007577:JC Virus / Q000145:classification / Q000254:growth & development / Q000302:isolation & purification*; D007968:Leukoencephalopathy, Progressive Multifocal / Q000453:epidemiology; D008954:Models, Biological; D009103:Multiple Sclerosis / Q000821:virology; D009369:Neoplasms / Q000821:virology; D027601:Polyomavirus Infections / Q000453:epidemiology