Article
Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations.
Journal of neuropathology and experimental neurology - 1 Jan 2001
Zervas M, Dobrenis K, Walkley S U
Abstract excerpt
Niemann-Pick disease type C (NPC) is a lethal neurologic storage disorder of children most often caused by a defect in the protein NPC1. To better understand the disease we thoroughly characterized the cellular and morphological alterations occurring in murine, feline, and human NPC. Using immuno...
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