The cholesterol storage disorder of the mutant BALB/c mouse. A primary genetic lesion closely linked to defective esterification of exogenously derived cholesterol and its relationship to human type C Niemann-Pick disease

An inborn murine cholesterol storage disorder exists which is characterized by a lesion in intracellular cholesterol esterification not accounted for by any discernible abnormality in acyl-CoA: cholesterol acyltransferase (Pentchev, P.G., Boothe, A.D., Kruth, H.S., Weintroub, H., Stivers, J., and Br...

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Veröffentlicht in:	The Journal of biological chemistry 1986-02, Vol.261 (6), p.2772-2777
Hauptverfasser:	Pentchev, P G, Comly, M E, Kruth, H S, Patel, S, Proestel, M, Weintroub, H
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Cholesterol Esters - metabolism Desmosterol - metabolism Disease Models, Animal Fibroblasts - metabolism Heterozygote Homozygote Lipoproteins, LDL - metabolism Mevalonic Acid - metabolism Mice Mice, Inbred BALB C - genetics Mutation Niemann-Pick Diseases - genetics Squalene - metabolism
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