Publication: Aberrant expressions of delta-protocadherins
in the brain of Npc1 mutant mice
Authors
Yan, Xin ; Lukas, Jan ; Lin, Juntang ; Ernst, Mathias ; Koczan, Dirk ; Witt, Martin ; Fuellen, Georg ; Wree, Andreas ; Rolfs, Arndt ; Luo, Jiankai
item.page.secondaryauthor
item.page.director
Publisher
F. Hernández y Juan F. Madrid. Universidad de Murcia: Departamento de Biología Celular e Histología
publication.page.editor
publication.page.department
Description
Abstract
Niemann-Pick type C1 (NPC1) disease is an
autosomal recessive disorder characterized by
dysmyelination and neurodegeneration, which can result
in the death of patients in early childhood in some cases.
Members of the delta-protocadherins (Pcdhs) play
important roles in neurogenesis and brain development.
In this study, we compared expression profiles of Pcdhs
in the brain between wild-type and Npc1 mutant mice
from postnatal day (P) 9 onwards by in situ
hybridization. Our data show that laminar distribution of
some Pcdhs in the cerebral cortex of Npc1 mutated mice
is different from that of wild-type mice. Furthermore,
expressions of Pcdhs by oligodendrocytes in the corpus
callosum and by Purkinje cells and granular cells in the
cerebellum are strongly decreased in Npc1 mutated mice
at later stages. Taken together, our data suggest that
aberrant expression of Pcdhs is a pathological process
accompanied by neurodegeneration in Npc1 mutant
mice.
publication.page.subject
Citation
item.page.embargo
Ir a Estadísticas
Este ítem está sujeto a una licencia Creative Commons. Licencia Creative Commons