COCULTIVATION OF NIEMANN-PICK DISEASE TYPE-C FIBROBLASTS BELONGING TOCOMPLEMENTATION GROUP-ALPHA AND GROUP-BETA STIMULATES LDL-DERIVED CHOLESTEROL ESTERIFICATION

Niemann-Pick disease type C (NPC) is a neurovisceral storage disorder with an unknown primary deficiency. Somatic cell hybridization experim ents using human cultured fibroblasts have shown that two complementat ion groups (NPC-alpha and NPC-beta) are associated with the biochemica l and clinical phenotypes comprising NPC. We identified the rarer comp lementation group NPC-beta originally using the technique of filipin s taining as a marker for complementation. In this study we show that th e esterification of cholesterol derived from the LDL pathway can be us ed as an isotopic assay. However, multinuclear hybrids exhibit a delay ed induction in this pathway. Furthermore, we discovered that, in the presence of an LDL source, co-cultivation of fibroblasts belonging to NPC-alpha and NPC-beta stimulated cholesterol esterification.