Peer-reviewed veterinary case report
Degeneration of cholecystokinin-immunoreactive afferents to the VPL thalamus in a mouse model of Niemann-Pick disease type C.
- Journal:
- Brain research
- Year:
- 2004
- Authors:
- Ohara, Shinji et al.
- Affiliation:
- Department of Neurology · Japan
- Species:
- rodent
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.
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Search related cases →Original publication: https://pubmed.ncbi.nlm.nih.gov/15353235/