Article
Sphingosylphosphocholine effects on cultured astrocytes reveal mechanisms potentially involved in neurotoxicity in Niemann-Pick type A disease.
The European journal of neuroscience - 1 Aug 2007
Chiulli Nino, Codazzi Franca, Di Cesare Alessandra, Gravaghi Claudia, Zacchetti Daniele, Grohovaz Fabio
Abstract excerpt
Niemann-Pick type A is a disease characterized by the absence of a functional SMPD1 (acidic sphingomyelinase) gene and the abnormal accumulation of sphingomyelin. Under these conditions, also sphingosylphosphocholine (SPC, a sphingomyelin metabolite) accumulates in various tissues, including the brain, where it might act as a toxic stimulus, contributing to the appearance of the neurological symptoms. We studied...
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