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2014 ; 124
(12
): 5103-6
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An animal model for progressive multifocal leukoencephalopathy
#MMPMID25401466
Haley SA
; Atwood WJ
J Clin Invest
2014[Dec]; 124
(12
): 5103-6
PMID25401466
show ga
JC virus (JCV) causes progressive multifocal leukoencephalopathy (PML), a
demyelinating disease in humans. The disease, once considered fatal, is now
managed with immune reconstitution therapy; however, surviving patients remain
severely debilitated. Until now, there has been no animal model to study JCV in
the brain, and research into treatment has relied on cell culture systems. In
this issue of the JCI, Kondo and colleagues developed a mouse model in which
human glial cells are engrafted into neonatal mice that are both immunodeficient
and deficient for myelin basic protein. When challenged intracerebrally with JCV,
these mice exhibit some of the characteristics of PML. The establishment of this
chimeric mouse model is a significant advance toward understanding the mechanism
of JCV pathogenesis and the identification of drugs to treat or prevent the
disease.