The attenuated elevation of cytoplasmic calcium concentration following the uptake of low density lipoprotein in type C Niemann-Pick fibroblasts.
In Niemann-Pick disease type C fibroblasts, the deficiency of cholesterol esterification has been reported. In this experiment, we detected the attenuated elevation of cytoplasmic calcium concentration following low density lipoprotein uptake in the fibroblasts. Moreover, we administered calcium channel agonist (0.5 microM YC-170) and calcium channel antagonists to the fibroblasts. YC-170 improved the attenuated elevation of calcium concentration and the deficient cholesterol esterification by 40% and 90% respectively. Calcium channel antagonists decreased the cholesterol esterification in normal and affected fibroblasts. These data indicated that the attenuated elevation of cytoplasmic calcium concentration was strongly related to the etiology of this disease.
Yamamoto
T
T
Department of Pediatrics, Jikei University, Tokyo, Japan.
Tokoro
T
T
Eto
Y
Y
D002118:Calcium / Q000378:metabolism*; D002120:Calcium Channel Agonists / Q000494:pharmacology; D002121:Calcium Channel Blockers; D002478:Cells, Cultured; D008078:Cholesterol, LDL / Q000378:metabolism; D003593:Cytoplasm / Q000378:metabolism*; D000078404:Epidermal Cells; D005347:Fibroblasts / Q000187:drug effects / Q000378:metabolism*; D006801:Humans; D008077:Lipoproteins, LDL / Q000378:metabolism* / Q000494:pharmacology; D009542:Niemann-Pick Diseases / Q000145:classification / Q000378:metabolism*; D009543:Nifedipine / Q000031:analogs & derivatives / Q000494:pharmacology; D019301:Oleic Acid; D009829:Oleic Acids / Q000378:metabolism
D002120:Calcium Channel Agonists; D002121:Calcium Channel Blockers; D008078:Cholesterol, LDL; D008077:Lipoproteins, LDL; D009829:Oleic Acids; D019301:Oleic Acid; C046721:YC 170; D009543:Nifedipine; D002118:Calcium