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Publication : The mouse rostral cerebellar malformation gene encodes an UNC-5-like protein.

First Author  Ackerman SL Year  1997
Journal  Nature Volume  386
Issue  6627 Pages  838-42
PubMed ID  9126743 Mgi Jnum  J:40243
Mgi Id  MGI:87586 Doi  10.1038/386838a0
Citation  Ackerman SL, et al. (1997) The mouse rostral cerebellar malformation gene encodes an UNC-5-like protein. Nature 386(6627):838-42
abstractText  Migration of neurons from proliferative zones to their functional sites is fundamental to the normal development of the central nervous system(1,2). Mice homozyous for the spontaneous rostral cerebellar malformation mutation (rcm(s)) or a newly identified transgenic insertion allele (rcm(tg)) exhibit cerebellar and midbrain defects, apparently as a result of abnormal neuronal migration. Laminar structure abnormalities in lateral regions of the rostral cerebellar cortex have been described in homozygous rcm(5)-mice(3). We now demonstrate that the cerebellum of both rcm(5) and rcm(tg) homozygotes is smaller and has fewer folia than in the wild-type, ectopic cerebellar cells are present in midbrain regions by three days after birth, and there are abnormalities in postnatal cerebellar neuronal migration. We have cloned the rcm complementary DNA, which encodes a transmembrane receptor of the immunoglobulin superfamily. The sequence of the rcm protein (Rcm) is highly similar to that of UNC-5, a Caenorhabditis elegans protein that is essential for dorsal guidance of pioneer axons and for the movement of cells away from the netrin ligand, which is encoded by the unc-6 gene(4-7). As Rcm is a member of a newly described family of vertebrate homologues of UNC-5 which are netrin- binding proteins, our results indicate that UNC-5-like proteins may have a conserved function in mediating netrin- guided migrations(8).
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