Normalization of liver glucosylceramide levels in the "Gaucher" mouse by phosphatidylserine injection.
A model of the human genetic disorder, Gaucher disease, can be rapidly generated in mice by the injection of emulsified glucosylceramide and an inhibitor of the lipid's hydrolase, conduritol B epoxide. The liver grows rapidly as it absorbs the load of lipid but the effect disappears as new glucosidase is formed and the load is hydrolyzed. This normalization process is accelerated by treatment with phosphatidylserine, which is a known stimulator of the enzyme. It is possible that injecting the phospholipid into Gaucher patients would have a therapeutic effect since it might help them utilize their residual glucosidase to destroy stored glycolipid.
Datta
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Mental Health Research Institute, University of Michigan, Ann Arbor 48109.
Radin
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D000818:Animals; D001835:Body Weight / Q000187:drug effects; D002554:Cerebrosides / Q000378:metabolism*; D004195:Disease Models, Animal; D005776:Gaucher Disease / Q000378:metabolism*; D005962:Glucosylceramidase / Q000378:metabolism; D005963:Glucosylceramides / Q000378:metabolism* / Q000494:pharmacology; D008099:Liver / Q000187:drug effects / Q000378:metabolism*; D051379:Mice; D009929:Organ Size / Q000187:drug effects; D010718:Phosphatidylserines / Q000494:pharmacology*
D002554:Cerebrosides; D005963:Glucosylceramides; D010718:Phosphatidylserines; D005962:Glucosylceramidase