JC virus (JCV) is a ubiquitous human papovavirus and is strongly associated with the demyelinating disease, progressive multifocal leukoencephalopathy (PML). PML occurs in patients who are immunosuppressed by illness, immunosuppressive therapy or genetic disorders. JCV exhibits a highly specific host range and tissue specificity. In immunosuppressed humans, viral particles are detected in brain cells of glial origin, specifically oliodendrocytes and astrocytes. It is the intent of this study to determine if introduction of JCV into transgenic mice would provide an animal model to study these human diseases. Transgenic mice have been produced containing JC virus early region genes under the control of the JCV promoter/enhancer element. Five mice were obtained containing the JCV sequences. Three female founder mice succumbed to tumors, resulting from metastasis of an adrenal medullary neuroblastoma. Two of five mice produced offspring which developed a neurological disorder related to a myelin deficiency. Neuropathological analysis indicated a myelin deficiency in the central nervous system apparently correlated with the expression of JCV T-antigen in brain tissue.
