Journal of Lipid Research, Vol 35, 1839-1849, Copyright © 1994 by Lipid Research, Inc.

ARTICLES

A second complementation class of cholesterol transport mutants with a variant Niemann-Pick type C phenotype

NK Dahl, MA Daunais and L Liscum
Department of Physiology, Tufts University School of Medicine, Boston, MA 02111.

We previously isolated Chinese Hamster ovary cell mutants that were defective in the intracellular transport of low density lipoprotein (LDL)-derived cholesterol (Dahl, N.K., K.L. Reed, M.A. Daunais, J.R. Faust, and L. Liscum. 1992 J. Biol. Chem. 267: 4889-4896). Several of the mutants exhibited the same biochemical phenotype as classical Niemann-Pick type C (NPC) fibroblasts. Complementation analysis between these mutants and other cholesterol transport mutants with a variant biochemical phenotype has defined two complementation classes. One class is characterized by expression of the classical NPC phenotype and may represent a true cholesterol transport mutant, while the second is characterized by expression of a variant NPC phenotype and may represent a signaling defect in LDL-sensitive homeostatic responses.
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