Degeneration of cholecystokinin-immunoreactive afferents to the VPL thalamus in a mouse model of Niemann-Pick disease type C

Brain Res. 2004 Oct 1;1022(1-2):244-6. doi: 10.1016/j.brainres.2004.06.037.

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.

Publication types

  • Comparative Study

MeSH terms

  • Afferent Pathways / metabolism*
  • Animals
  • Axons / metabolism
  • Axons / ultrastructure
  • Cholecystokinin / metabolism*
  • Disease Models, Animal
  • Immunohistochemistry / methods
  • Intracellular Signaling Peptides and Proteins
  • Mice
  • Mice, Inbred BALB C
  • Mice, Mutant Strains
  • Microscopy, Immunoelectron / methods
  • Nerve Degeneration / metabolism*
  • Nerve Degeneration / physiopathology
  • Neurons / metabolism
  • Neurons / pathology
  • Neurons / ultrastructure
  • Niemann-Pick C1 Protein
  • Niemann-Pick Diseases / metabolism*
  • Niemann-Pick Diseases / physiopathology*
  • Proteins / genetics
  • Ventral Thalamic Nuclei / metabolism*
  • Ventral Thalamic Nuclei / pathology
  • Ventral Thalamic Nuclei / ultrastructure

Substances

  • Intracellular Signaling Peptides and Proteins
  • Niemann-Pick C1 Protein
  • Npc1 protein, mouse
  • Proteins
  • Cholecystokinin