Wednesday, December 7, 2016

Sphingosine and Calcium Defect Causes Niemann Pick Type C, Cholesterol Downstream Effect Of The Disease

October 28, 2008 by  
Filed under NPC News

In a recent issue of Nature Medicine, Oxford University reports that Niemann Pick Type C disease is a sphingosine storage disease that causes deregulation of lysosomal calcium.  According to Oxford researchers, there is a block in the late endosome to lysosome transport in NPC disease, resulting in the downstream storage of cholesterol and glycosphingolipids (a lipid derived from a ceramide that contains a carbohydrate such as glucose or galactose).

For the lay person, this means that cholesterol is not the ultimate culprit in Niemann Pick Type C disease — there is a cascade of events happening in the cell with sphingosine using up the calcium in the cell which then triggers a cholesterol traffic jam in the cells of NPC patients. Oxford conducted extensive studies and published a paper which shows many complex experiments.One experiment showed that Curcumin is helping to correct the loss of calcium and rejuvenate the calcium in the cell.

Typically, NPC mice die at 13 weeks of age.  Above is a picture of a 9 week old Niemann Pick Type C mouse — the one on the top is not treated with Curcumin, the one on the bottom has been treated with Curcumin.  Curcumin is obviously extending its life.  Curcumin is considerd a "spice or supplement," not classified as a drug. This is amazing research by Oxford and I am grateful for their research as my 4 year old identical twin girls, Addi and Cassi, are on high doses of bioavailable Curcumin (delivered to them 2x a day in applesauce!)

The data from Oxford suggests we must pursue a new clinical intervention strategy for treating Niemann Pick Type C, this horrible neurological disorder that is trying to take the lives of my precious twins. What Oxford has also shown us is that NPC patients potentially have another "treatment option" available to them in addition to Zavesca. For many NPC patients who have no access to drugs of any kind, Curcumin could help their suffering children.  Non-toxic drugs similar to myriocin that reduce sphingosine levels could be a therapeutic targets for NPC patients or drugs that act to reguvinate the calcium in the cell could help as well.

If you have a brain disease and have been told that Curcumin can help you and are interested in obtaining the bioavailable Curcumin UCLA Alzheimer’s reseachers have developed for humans, contact Verdure Sciences.  As I reported in my last blog, Verdure Sciences has a compound of bioavailable Curcumin that crosses the formidable blood brain barrier in humans.

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  1. [...] been treated with Curcumin and Curcumin is extending its life. Whoa! Typically, NPC mice will die Read More|||Curcumin Inhibits Glyoxalase 1—A Possible Link to Its Anti-Inflammatory and Anti-Tumor Activity [...]



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