title: Degeneration of cholecystokinin-immunoreactive afferents to the VPL thalamus in a mouse model of Niemann-Pick disease type C.
abstract: Niemann-Pick disease type C (NP-C) is a progressive neurological disorder of lipid metabolism. The Balb/C npc1 mutant strain is a genetically authentic murine model of NPC, which reproduce the clinical and histologic features of human NP-C. In the present study, we show that cholecystokinin (CCK)-immunoreactive fibers in the thalamic VPL nuclei, which are densely distributed in controls, degenerate in NPC mice. This degeneration is associated with the appearance of CCK-immunoreactive axonal spheroids containing characteristic intracellular inclusions of NP-C. These observations provide supportive evidence of the occurrence of dying-back axonopathy of neurons in the dorsal column nuclei in this mouse model.
authors.affiliation: Department of Neurology, National Chushin-Matsumoto Hospital, 811 Kotobuki, Matsumoto 399-0021, Japan. neuro@cameo.plala.or.jp
authors.forename: Shinji
authors.initials: S
authors.lastname: Ohara
authors.forename: Yoko
authors.initials: Y
authors.lastname: Ukita
authors.forename: Haruaki
authors.initials: H
authors.lastname: Ninomiya
authors.forename: Kousaku
authors.initials: K
authors.lastname: Ohno
mesh_terms: D000344:Afferent Pathways / Q000378:metabolism*; D000818:Animals; D001369:Axons / Q000378:metabolism / Q000648:ultrastructure; D002766:Cholecystokinin / Q000378:metabolism*; D004195:Disease Models, Animal; D007150:Immunohistochemistry / Q000379:methods; D047908:Intracellular Signaling Peptides and Proteins; D051379:Mice; D008807:Mice, Inbred BALB C; D008817:Mice, Mutant Strains; D016253:Microscopy, Immunoelectron / Q000379:methods; D009410:Nerve Degeneration / Q000378:metabolism* / Q000503:physiopathology; D009474:Neurons / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D000077296:Niemann-Pick C1 Protein; D009542:Niemann-Pick Diseases / Q000378:metabolism* / Q000503:physiopathology*; D011506:Proteins / Q000235:genetics; D020651:Ventral Thalamic Nuclei / Q000378:metabolism* / Q000473:pathology / Q000648:ultrastructure
chemical_list: D047908:Intracellular Signaling Peptides and Proteins; D000077296:Niemann-Pick C1 Protein; C106831:Npc1 protein, mouse; D011506:Proteins; D002766:Cholecystokinin