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

Susan L. Ackerman, Leslie P. Kozak, Stefan A. Przyborski, Laurie A. Rund, Bert Boyer, Barbara B. Knowles

Research output: Contribution to journalArticle

299 Citations (Scopus)

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) 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 mice. We now demonstrate that the cerebellum of both rcm 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 netringuided migration.

Original languageEnglish (US)
Pages (from-to)838-842
Number of pages5
JournalNature
Volume386
Issue number6627
DOIs
StatePublished - Apr 24 1997
Externally publishedYes

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Mutation
Genes
Proteins
Mesencephalon
Caenorhabditis elegans Proteins
Cerebellar Cortex
Homozygote
Cerebellum
Cell Movement
Vertebrates
Immunoglobulins
Carrier Proteins
Central Nervous System
Complementary DNA
Alleles
Parturition
Ligands
Neurons

ASJC Scopus subject areas

  • General

Cite this

Ackerman, S. L., Kozak, L. P., Przyborski, S. A., Rund, L. A., Boyer, B., & Knowles, B. B. (1997). The mouse rostral cerebellar malformation gene encodes an UNC-5-like protein. Nature, 386(6627), 838-842. https://doi.org/10.1038/386838a0

The mouse rostral cerebellar malformation gene encodes an UNC-5-like protein. / Ackerman, Susan L.; Kozak, Leslie P.; Przyborski, Stefan A.; Rund, Laurie A.; Boyer, Bert; Knowles, Barbara B.

In: Nature, Vol. 386, No. 6627, 24.04.1997, p. 838-842.

Research output: Contribution to journalArticle

Ackerman, SL, Kozak, LP, Przyborski, SA, Rund, LA, Boyer, B & Knowles, BB 1997, 'The mouse rostral cerebellar malformation gene encodes an UNC-5-like protein', Nature, vol. 386, no. 6627, pp. 838-842. https://doi.org/10.1038/386838a0
Ackerman SL, Kozak LP, Przyborski SA, Rund LA, Boyer B, Knowles BB. The mouse rostral cerebellar malformation gene encodes an UNC-5-like protein. Nature. 1997 Apr 24;386(6627):838-842. https://doi.org/10.1038/386838a0
Ackerman, Susan L. ; Kozak, Leslie P. ; Przyborski, Stefan A. ; Rund, Laurie A. ; Boyer, Bert ; Knowles, Barbara B. / The mouse rostral cerebellar malformation gene encodes an UNC-5-like protein. In: Nature. 1997 ; Vol. 386, No. 6627. pp. 838-842.
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