title: Aberrant expressions of delta-protocadherins in the brain of Npc1 mutant mice.
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 of both 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.
authors.affiliation: Albrecht-Kossel-Institute for Neuroregeneration, Rostock University Medical Center, Rostock, Germany.
authors.forename: Xin
authors.initials: X
authors.lastname: Yan
authors.affiliation: Albrecht-Kossel-Institute for Neuroregeneration, Rostock University Medical Center, Rostock, Germany.
authors.forename: Jan
authors.initials: J
authors.lastname: Lukas
authors.affiliation: Institute of Anatomy I, School of Medicine University of Jena, Jena, Germany.
authors.forename: Juntang
authors.initials: J
authors.lastname: Lin
authors.affiliation: Institute for Biostatistics and Informatics in Medicine and Ageing Research, University of Rostock, Rostock, Germany.
authors.forename: Mathias
authors.initials: M
authors.lastname: Ernst
authors.affiliation: Institute for Immunology, Rostock University Medical Center, Rostock, Germany.
authors.forename: Dirk
authors.initials: D
authors.lastname: Koczan
authors.affiliation: Institute of Anatomy, Rostock University Medical Center, Rostock, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Witt
authors.affiliation: Institute for Biostatistics and Informatics in Medicine and Ageing Research, University of Rostock, Rostock, Germany.
authors.forename: Georg
authors.initials: G
authors.lastname: Fuellen
authors.affiliation: Institute of Anatomy, Rostock University Medical Center, Rostock, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Wree
authors.affiliation: Albrecht-Kossel-Institute for Neuroregeneration, Rostock University Medical Center, Rostock, Germany.
authors.forename: Arndt
authors.initials: A
authors.lastname: Rolfs
authors.affiliation: Albrecht-Kossel-Institute for Neuroregeneration, Rostock University Medical Center, Rostock, Germany. jiankai.luo@uni-rostock.de.
authors.forename: Jiankai
authors.initials: J
authors.lastname: Luo
mesh_terms: D000818:Animals; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism* / Q000473:pathology*; D015820:Cadherins / Q000032:analysis / Q000096:biosynthesis*; D004195:Disease Models, Animal; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D047908:Intracellular Signaling Peptides and Proteins; D051379:Mice; D008817:Mice, Mutant Strains; D009154:Mutation; D000077296:Niemann-Pick C1 Protein; D052556:Niemann-Pick Disease, Type C / Q000378:metabolism* / Q000473:pathology; D020411:Oligonucleotide Array Sequence Analysis; D011506:Proteins / Q000235:genetics*
chemical_list: D015820:Cadherins; D047908:Intracellular Signaling Peptides and Proteins; D000077296:Niemann-Pick C1 Protein; C106831:Npc1 protein, mouse; D011506:Proteins