Absence of the basilar pons in mice lacking a functional Large glycosyltransferase gene suggests a defect in pontine neuron migration.
Absence of the basilar pons in mice lacking a functional Large glycosyltransferase gene suggests a defect in pontine neuron migration.
复制标题
缺乏功能性大糖基转移酶基因的小鼠中基底脑桥的缺失表明脑桥神经元迁移存在缺陷。
DOI：
10.1016/j.brainres.2006.08.008
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发表时间：
2006
期刊：
影响因子：
2.9
通讯作者：
Mallott,JacobM
中科院分区：
文献类型：
--
作者：
Litwack,EDavid;Lee,Yongsuk;Mallott,JacobM
Several forms of congenital muscular dystrophy result from mutations in glycosyltransferases that modify α-dystroglycan. As pontine hypoplasia has been reported in some clinical cases of congenital muscular dystrophy, we have begun to examine whether these glycosyltransferases are required for the normal development of the basilar pons, one of several precerebellar nuclei of the hindbrain. In veils (Largevls) mice, which carry a loss-of-function mutation in the Large glycosyltransferase gene, the basilar pons is absent. Instead, ectopic clusters of pontine neurons are found lateral to their normal site, suggesting that these neurons are unable to migrate to their appropriate site. Two other precerebellar nuclei, the lateral reticular nucleus and the inferior olive, are present in Largevlsmice. In addition, the basilar pons forms normally in dystrophin-deficient mice. These results demonstrate that the Large glycosyltransferase but not dystrophin is required for normal basilar pontine development.
