Faculty Research 1990 - 1999
The mouse rostral cerebellar malformation gene encodes an UNC-5-like protein.
Document Type
Article
Publication Date
1997
Keywords
Animal, Blotting-Northern, Cell-Division: ph, Cell-Movement: ph, Cerebellum: ab, em, me, Cloning-Molecular, Gene-Expression, Helminth-Proteins, Homozygote, In-Situ-Hybridization, Membrane-Proteins, Mice, Mice-Inbred-C57BL, Mice-Transgenic, Molecular-Sequence-Data, Mutation, Nerve-Growth-Factors: me, Neurons: ph, Polymerase-Chain-Reaction, Receptors-Growth-Factor, Receptors-Nerve-Growth-Factor: ge, ph, Sequence-Homology-Amino-Acid, SUPPORT-U-S-GOVT-P-H-S, Tissue-Distribution
First Page
838
Last Page
842
JAX Source
Nature 1997 Apr 24;386(6627):838-42
Abstract
Migration of neurons from proliferative zones to their functional sites is fundamental to the normal development of the central nervous system. Mice homozygous 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(s) mice. We now demonstrate that the cerebellum of both rcm(s) 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. 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 migration.
Recommended Citation
Ackerman SL,
Kozak LP,
Przyborski SA,
Rund LA,
Boyer BB,
Knowles BB.
The mouse rostral cerebellar malformation gene encodes an UNC-5-like protein. Nature 1997 Apr 24;386(6627):838-42