Mutation Repairs Retrovirus Damage in Mouse Model

The investigators studied a line of mice known as "vibrator” that have severe tremors, progressive degeneration of the brain stem and spinal cord, and premature death. Vibrator mice that carried the mvb1 gene (Modifier-of-vibrator-1, which reduces the damaging characteristics of the vibrator neurologic gene mutation caused by insertion of the mouse IAP retrovirus) showed reduced tremor severity and survived to adulthood. Mvb1 raised the amount of normal RNA made from the mutant vibrator gene.

Mvb1 was also found to partially alleviate the Eya1 mutation that is a mouse model of human branchio-otorenal syndrome, which is also caused by an IAP retrovirus. The investigators further reported in the September 28, 2003, issue of Nature Genetics that positional complementation showed that Mvb1 is part of the mRNA nuclear export factor Nxf1.

"The properties of this gene could be used to engineer a system for controlling some mutations caused by retroviruses,” said senior author Dr. Bruce Hamilton, assistant professor of medicine at the University of California, San Diego. "This could be particularly useful for creating mouse models of human disorders where loss of gene expression is a target of therapeutic efforts, but the dose-responsiveness required for functional recovery is unknown, such as in Fragile X syndrome and certain cancers.”




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