Article
Dysregulation of protein modification by ISG15 results in brain cell injury.
Genes & development - 1 Sept 2002
Ritchie Kenneth J, Malakhov Michael P, Hetherington Christopher J, Zhou Liming, Little Marie-Terese, Malakhova Oxana A, Sipe Jack C, Orkin Stuart H, Zhang Dong-Er
Abstract excerpt
UBP43 (USP18) is a protease that removes the ubiquitin-like modifier ISG15 from conjugated proteins. Here we present the first report of dysregulation of protein ISG15 modification by the generation of UBP43 knockout mice. In the absence of UBP43, brain tissue showed an elevated level of ISG15 conjugates, and cellular necrosis was evident in the ependyma. Such disruption of the blood-brain barrier resulted in...
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