Binding, internalization, and hydrolysis of low density lipoprotein in long-term lymphoid cell lines from a normal subject and a patient with homozygous familial hypercholesterolemia.
Long-term established human lymphoid cells were shown to possess high affinity cell surface receptors for low density lipoprotein (LDL), the major cholesterol-carrying protein in human plasma. Binding of LDL to these receptors was followed by internalization of the lipoprotein and hydrolysis of its protein and cholesteryl ester components. Cultured lymphocytes from a patient with the homozygous form of familial hypercholesterolemia lacked cell surface LDL receptors and therefore failed to take up and degrade the lipoprotein with high affinity. Cultured human lymphocytes should prove useful for further studies of: (a) the relation between cholesterol metabolism and cellular function and (b) the mechanism by which LDL binding at the cell surface leads to internalization of the lipoprotein.
D001059:Apoproteins / Q000378:metabolism; D001665:Binding Sites; D001692:Biological Transport; D002460:Cell Line; D002784:Cholesterol / Q000378:metabolism; D003208:Concanavalin A / Q000378:metabolism; D006720:Homozygote; D006801:Humans; D006868:Hydrolysis; D006937:Hypercholesterolemia / Q000235:genetics / Q000378:metabolism*; D008075:Lipoproteins, HDL / Q000378:metabolism; D008077:Lipoproteins, LDL / Q000378:metabolism*; D008214:Lymphocytes / Q000378:metabolism*; D011955:Receptors, Drug
D001059:Apoproteins; D008075:Lipoproteins, HDL; D008077:Lipoproteins, LDL; D011955:Receptors, Drug; D003208:Concanavalin A; D002784:Cholesterol