Similar content of phospholipids and gangliosides in normal and homozygous familial hypercholesterolemia fibroblasts.

Abstract:

:The cellular content of total and individual phospholipids and gangliosides was measured in fibroblasts cultured from four normal subjects, three patients with lysosomal lipid storage diseases, and two subjects with homozygous familial hypercholesterolemia. Measurements were made on cells grown in medium containing fetal calf serum under conditions in which normal cells derive cholesterol for cell growth from low density lipoprotein present in the fetal calf serum, whereas familial hypercholesterolemia homozygote cells, which lack cell surface low density lipoprotein receptors, derive cholesterol from endogenous synthesis. No difference was observed in the cellular content of total or individual phospholipids and gangliosides in the normal and familial hypercholesterolemia homozygote cells. In contrast, cells from a patient with Niemann-Pick disease and a patient with Sandhoff disease showed elevations in the content of sphingomyelin and complex gangliosides, respectively.

journal_name

J Lipid Res

authors

Fishman PH,Bradley RM,Brown MS,Faust JR,Goldstein JL

subject

Has Abstract

pub_date

1978-03-01 00:00:00

pages

304-8

issue

3

eissn

0022-2275

issn

1539-7262

journal_volume

19

pub_type

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