Scientists have identified a gene in mice that is necessary for normal brain development and may contribute to the most common form of primary brain tumors in children.
Dr. Valeri Vasioukhin and colleagues at the Fred Hutchinson Cancer Research Center have discovered that a gene known as "lethal giant larvae 1" (a.k.a. Lgl1) plays a critical role in shaping cell behavior during embryonic brain development. Lgl1 was initially identified in the fruit fly Drosophila, where it regulates cell polarity (the overall directionality of a cell) as well as cell proliferation. Dr. Vasioukhin and colleagues now show a similarly important role for Lgl1 in the mammalian brain.
To gain insight into Lgl1 function in mammals, Dr. Vasioukhin and colleagues generated mice specifically lacking the Lgl1 gene. These Lgl1-knockout mice – as they are known – developed normally at first, but by day 12.5 of gestation exhibited dramatic abnormalities. Lgl1-mutant pups have a dome-shaped head, severe hydrocephaly and die within 24 hours after birth. Internally, there is an expansion of the striatum region of the brain, along with the formation of abnormal cell groupings called rosettes.
Heather Cosel | EurekAlert!
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