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Could you check my answer, they should be from this article.

(Cholesterol Is Sequestered in the Brains of Mice with Niemann-Pick Type C Disease but Turnover Is Increased) writing by Xie 2000.


You can find it in the google



1.    What is the main pathology of human NPC1 disease?


Niemann Pick type C disease (NPC) is a sphingolipid storage disorder that results from inherited deficiencies of intracellular lipid trafficking proteins, and is characterized by an accumulation of cholesterol and glycosphingolipids in late endosomes and lysosomes.it is postulated that NPC1 regulates retrograde transport of multiple lysosomal cargoes in the late endosomal/lysosomal pathway, NPC1 and NPC2 function cooperatively, thus NPC1 has homology to other proteins involved in cholesterol homeostasis, such as patched, HMG CoA reductase and SCAP


2.     What is the function of NPC1 in cholesterol metabolism?


Both NPC1 and NPC2 are required for transporting cholesterol out of lysosome after lipoprotein uptake. Mechanism not understood.

Fig.2

3.     What is the main abnormality of NPC1-/- in brain observed by histology?


loss of Purkinje cells in the NPC2/2 animals


Fig. 3A, Table 1

4.     Cholesterol in myelin decreased in 7-week-old NPC-/- mice, whereas total cholesterol in various brain regions did not decrease as much as in myelin. Where would the rest of cholesterol be in the  NPC-/- mice?

the cholesterol accumulates in the cells of the CNS, as in all other tissues of the body, this sequestration, and the accompanying neurodegeneration and apparent loss

of myelin,

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