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A more recent version of this article appeared on March 1, 2006

Papers In Press, published online ahead of print December 10, 2005
J. Lipid Res., doi:10.1194/jlr.M500482-JLR200
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Submitted on November 3, 2005
Revised on December 6, 2005
Accepted on December 9, 2005

The Niemann-Pick C1 protein in recycling endosomes of pre-synaptic nerve terminals

Barbara Karten, Robert B. Campenot, Dennis E. Vance, and Jean E. Vance

Medicine Dept., University of Alberta, Edmonton, AB T6G 2S2

Corresponding Author: jean.vance{at}ualberta.ca

Niemann-Pick Type C disease is a fatal, neurodegenerative disorder caused in 95% of cases by loss of function of NPC1, a ubiquitous endosomal transmembrane protein. A biochemical hallmark of NPC-deficiency is cholesterol accumulation in the endocytic pathway. Although cholesterol trafficking defects are observed in all cell types, neurons are the most vulnerable to NPC1-deficiency, suggesting a specialized function for NPC1 in neurons. We investigated the subcellular localization of NPC1 in neurons to gain insight into the mechanism of action of NPC1 in neuronal metabolism. We show that NPC1 is abundant in axons of sympathetic neurons, and is present in recycling endosomes in pre-synaptic nerve terminals. NPC1 deficiency causes morphological and biochemical changes in the pre-synaptic nerve terminal. Synaptic vesicles from Npc1-/- mice have normal cholesterol content but altered protein composition. We propose that NPC1 plays a previously unrecognized role in the pre-synaptic nerve terminal and that NPC1 deficiency at this site might contribute to the progressive neurological impairment in NPC disease.


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