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Changes that occur in the flux of cholesterol out of the central nervous system (CNS)
in one form of neurodegeneration, Niemann-Pick type C disease.
In this syndrome, there is loss of large neurons such as Purkinje cells and partial
demyelination. The excess sterol released by these degenerative processes must be mobilized
and excreted across the blood brain barrier as either 24(S)-hydroxycholesterol(24-C-OH)
or possibly as cholesterol itself [C (?)]. Both microglial cells and apolipoprotein E
(apoE) may play important roles in this excretory process.
(See Dietschy and Turley, p.
1375.)
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Copyright © 2005 by the American Society for Biochemistry and Molecular Biology.