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J. Cell Biol.,
Volume 143, Number 5, November 30, 1998 1305-1315
/
) Mice Suggest
Functional Overlap between the Cell Adhesion Molecule L1
and 440-kD AnkyrinB in Premyelinated Axons

* Howard Hughes Medical Institute and *Departments of Cell Biology, *Biochemistry, The L1 CAM family of cell adhesion molecules and the ankyrin family of spectrin-binding proteins are candidates to collaborate in transcellular complexes used in diverse contexts in nervous systems of
vertebrates and invertebrates. This report presents evidence for functional coupling between L1 and 440-kD
ankyrinB in premyelinated axons in the mouse nervous
system. L1 and 440-kD ankyrinB are colocalized in premyelinated axon tracts in the developing nervous system and are both down-regulated after myelination. AnkyrinB (
Neurobiology,
Anesthesiology,
and
Radiology, Duke University Medical Center, Durham, North Carolina 27710
/
) mice exhibit a phenotype similar to,
but more severe, than L1 (
/
) mice and share features of human patients with L1 mutations. AnkyrinB
(
/
) mice exhibit hypoplasia of the corpus callosum
and pyramidal tracts, dilated ventricles, and extensive degeneration of the optic nerve, and they die by postnatal day 21. AnkyrinB (
/
) mice have reduced L1 in
premyelinated axons of long fiber tracts, including the
corpus callosum, fimbria, and internal capsule in the
brain, and pyramidal tracts and lateral columns of the
spinal cord. L1 was evident in the optic nerve at postnatal day 1 but disappeared by postnatal day 7 in mutant mice while NCAM was unchanged. Optic nerve axons
of ankyrinB (
/
) mice become dilated with diameters
up to eightfold greater than normal, and they degenerated by day 20. These findings provide the first evidence for a role of ankyrinB in the nervous system and
support an interaction between 440-kD ankyrinB and
L1 that is essential for maintenance of premyelinated
axons in vivo.
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