Term
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Definition
polyomaviridiae, along with BK virus and Simian Virus 40 |
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Term
JCV is the opportunistic infectious agent of the fatal demyelinating disease ________. |
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Definition
progressive multifocal leukoencephalopathy |
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Term
What percent of the population has JCV? |
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Definition
80%, infection is thought to be subclinical and occur in early childhood |
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Term
What percent of HIV-1 infected patients will exhibit neurological disorders? what percent will develop PML? |
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Definition
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Term
What are the symptoms of PML? |
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Definition
multiple neurologic signs and symptoms reflecting the multifocal areas of demyelination: motor weakness, visual disturbances including double vision, subcortical dementia, slowed motor function, limb paralysis, rigidity and sensory disturbances. |
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Term
Where in the brain does PML develop? |
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Definition
white matter of any part of the CNS including the brainstem and the cerebellum although the cerebral hemispheres are most commonly involved. For unknown reasons, the optic nerves and spinal cord are virtually never involved (helps with differential diagnosis) |
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Term
What characterizes PML histopathologically? |
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Definition
multiple areas of demyelination with axonal preservation, located in the sub-cortical white matter. Contain several enlarged oligodendrocytes with intranuclear eosinophilic inclusion bodies accompanied by a reactive astrocytosis. Astrocytes might be enlarged an bizzare with irregularly lobulated hyperchromatic nuclei. Perivascular cuffs of lymphocytes and microglial nodules can be found |
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Term
Describe the JC virus genome: |
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Definition
fxnally divided into 3 regions; regulatory (noncoding) region (viral origin of DNA replication and bidirectional promotor), early coding region and a late coding region. |
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Term
Viral late genes of JCV encode... |
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Definition
structural capsid proteins (VP-1, VP-2, and VP-3), as well as the accessory protein Agno |
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Term
How does JCV destroy myelin sheath? |
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Definition
it infects the oligodendrocyte, replicates, lyses and destroys the oligodendrocyte and thereby the myelin sheath |
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Term
Describe the T antigen of JC virus. |
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Definition
79% homology with SV40 T antigen. Large. Nuclear localization signal, regions responsible for DNA binding, pol, helicase, ATPase activity. |
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Term
What is the most highly conserved domain among all polyomavirus T antigens? What is the significance of this? |
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Definition
ATPase domain which partially overlapse with the region important for complex formation with the cellulartumor suppressor protein, p53 |
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Term
Is T antigen an early or late protein? |
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Definition
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Term
What is the function of T-antigen? |
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Definition
multifunctional protein that contains ATPase and helicase activity and can bind to DNA, alpha polymerase and cell cycle regulators such as p53 and Rb |
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Term
What diseases does JCV cause in animal models? |
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Definition
tumors of neuroendocrine origin |
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Term
What is the only polyomavirus that induces tumor formation in non-humna primates? |
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Definition
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Term
T/F T antigen in teh absence of JC virus and therefore viral replication, can alter myelin formation to induce dysmelination. |
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Definition
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Term
Is JCV associated with human brian tumors? |
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Definition
there have been several reports of this |
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Term
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Definition
a member of the inhibitors of apoptosis family which is abundantly expressed during embryonic development in proliferating tissues, but is normally absent. There is increased expression in the intranuclear includsion bodies of JCV infected oligodendrocytes and teh cytoplasm of bizarre astrocytes. |
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