Low-density lipoprotein receptor, and

L. Maletinska, E.A. Blakely, K.A. Bjomstad, D.F. Deen, L.J. Knoff and T.M. Forte, Human glioblastoma cell lines levels of low-density lipoprotein receptor and low-density lipoprotein receptor-related protein, Cancer Res. 60 (2000) 2300-2303. 

R. Hamanaka, K. Kohno, T. Seguchi, K. Okamura, A. Morimoto, M. Ono, J. Ogata and M. Kuwano, Induction of low density lipoprotein receptor and a transcription factor SP-1 by tumor necrosis factor in human microvascular endothelial cells, J. Biol. Chem. 267 (1992) 13160-13165. 

Dehydrogenase Deficiency, Biotinidase Deficiency, and Adrenoleukodystrophy. Catabolism of essential amino acid skeletons is discussed in the chapters Phenylketonuria and HMG-CoA Lyase Deficiency. The chapters Inborn Errors of Urea Synthesis and Neonatal Hyperbilirubinemia discuss the detoxification and excretion of amino acid nitrogen and of heme. The chapter Gaucher Disease provides an illustration of the range of catabolic problems that result in lysosomal storage diseases. Several additional chapters deal with key aspects of intracellular transport of enzymes and metabolic intermediates the targeting of enzymes to lysosomes (I-Cell Disease), receptor-mediated endocytosis (Low-Density Lipoprotein Receptors and Familial Hypercholesterolemia) and the role of ABC transporters in export of cholesterol from the cell (Tangier disease). 

J. Nimpf and W. J. Schneider. 2000. From cholesterol transport to signal transduction Low density lipoprotein receptor, very low density lipoprotein receptor, and apolipoprotein E receptor-2 Biochim. Biophys. Acta 1529 287-298. (PubMed)... 

Noonan, D. M., FuUe, A., Valente, P, Cai, S., Horigan, E., Sasaki, M., Yamada, Y. and Hassell, J. R. (1991). The complete sequence of perlecan, a basement membrane heparan sulfate proteoglycan, reveals extensive similarity with laminin A chain, low density lipoprotein-receptor and the neural cell adhesion molecule. J. Biol. Chem. 266, 22939-22947. 

Trypanosoma cruzi expresses a neuraminidase activity which is developmentally regulated. Enzyme aetivity is maximal in infective trypomastigotes, 10 to 560 times less in epimastigotes, and is absent in amastigotes. The neuraminidase, of an apparent moleeular mass of 160-200 kDa, is located on the outer membrane and is released by a stage-specifie phospholipase C. Of interest is the presence of sequences similar to low-density lipoprotein receptor and to the type III module of fibronectin (14). 

Pereira, M. E. A., Mejia, J. S., Ortega-Barria, E., Matzilevich, D. and Prioli, R. P. (1991) The Trypanosoma cruzi neuraminidase contains sequences similar to bacterial neuraminidases, YWTD repeats of the low density lipoprotein receptor, and type III modules of fibronectin. 

A large protein family that contains extracellular domains homologous to EGF is called the EGF-domain proteins. EGF-like domains or modules are often found in many extracellular and membrane proteins (>70). They are involved in blood coagulation, fibrinolysis and the complement systems, matrix proteins, cell surface receptors, the low-density lipoprotein receptor and the developmentally important receptor. Notch. EGF-like domains may exist as multiple copies within proteins such as in the Drosophila protein Notch with 36 copies or as a single copy as in EGF itself. It can also be found in conjunction with other modules. 

Sachais BS, Kuo A, Nassar T, et al. Platelet factor 4 binds to low-density lipoprotein receptors and dismpts the endocytic machinery, resulting in retention of low-density lipoprotein on the cell surface. Blood 2002 99 3613-3622. 

Furbee JW Jr, Sawyer JK, Parks JS. Lecithin.cholesterol acyltransferase deficiency increases atherosclerosis in the low density lipoprotein receptor and apolipoprotein E knockout mice. J Biol Chem 2002 277 3511-3519. 

This autosomal dominant disorder is usually due to defective low density lipoprotein receptors and can lead to ischaemic heart disease in childhood... 

Very low-density lipoprotein receptor ( VLDLR) and APOE receptor-2 ( APOER2) are two gene family members with redundant functions. They are expressed in neurons of the developing brain and act as cell... 

BAUM J A, TENG H, ERDMAN J W Jr, WEIGEL R M, KLEIN B P, PERSKY V W, FREELS S, SURYA P, BAKHIT R M, RAMOS E, SHAY N F and POTTER s M (1998) Long-term intake of soy protein improves blood lipid profiles and increases mononuclear cell low-density-lipoprotein receptor messenger RNA in hypercolesterolemic postmenopausal women. Am J Clin Nutr. 68 (3) 545-51. 

Using human hepatoma-derived cell lines Kong et al. [268] showed that berberine increased mRNA and protein as well as the function of hepatic linear low density lipoprotein receptor (LDLR). It does not stimulate the transcription of LDLR, as the LDLR promoter activity was not increased by this compound. Post-transcriptional regulation appears to be the main working mechanism underlying the effect of this alkaloid on LDLR expression. It was proposed that berberine can be used as a monotherapy to treat hypercholes-terolemic patients [268]. Very recently it was observed [269] that berberine reduces cholesterol and Upid accumulations in plasma as well as Uver. 

Ishibashi S, Goldstein JL, Brown MS, Herz J, Bums DK. Massive xanthomatosis and atherosclerosis in cholesterol-fed low density lipoprotein receptor-negative mice. J Clin Invest 1994 93(5) 1885—1893. 

Lipoproteins. A lipoprotein is an endogenous macromolecule consisting of an inner apolar core of cholesteryl esters and triglycerides surrounded by a monolayer of phospholipid embedded with cholesterol and apoproteins. The functions of lipoproteins are to transport lipids and to mediate lipid metabolism. There are four main types of lipoproteins (classified based on their flotation rates in salt solutions) chylomicrons, very-low-density lipoprotein (VLDL), low-density lipoprotein (LDL), and high-density lipoprotein (HDL). These differ in size, molecular weight, and density and have different lipid, protein, and apoprotein compositions (Table 11). The apoproteins are important determinants in the metabolism of lipoproteins—they serve as ligands for lipoprotein receptors and as mediators in lipoproteins interconversion by enzymes. 

Tserentsoodol, N, Sztein, J, Campos, M, Gordiyenko, NV, Fariss, RN, Lee, JW, Fhesler, SJ, and Rodriguez, IR, 2006b. Uptake of cholesterol by the retina occurs primarily via a low density lipoprotein receptor-... 

Heath KE, Gahan M, Whittall RA, Humphries SE. Low-density lipoprotein receptor gene (LDLR) world-wide website in familial hypercholesterolaemia update, new features and mutation analysis. Atherosclerosis 2001 154 243-246. 

Vuorio AF, Ojala JP, Sarna S, Turtola H, Tikkanen MJ, Kontula K. Heterozygous familial hypercholesterolaemia the influence of the mutation type of the low-density-lipoprotein receptor gene and PvuII polymorphism of the normal allele on serum lipid levels and response to lo-vastatin treatment. J Intern Med 1995 237 43-48. 

Peltan, I. D., Thomas, A. V., Mikhailenko, I., Strickland, D. K., Hyman, B. T. and von Arnim, C. A. (2006). Fluorescence lifetime imaging microscopy (FLIM) detects stimulus-dependent phosphorylation of the low density lipoprotein receptor-related protein (LRP) in primary neurons. Biochem. Biophys. Res. Commun. 349, 24-30. 

Wade, D.P., Knight, B.F., and Soutar, A.K. (1985) Detection of the low-density lipoprotein receptor with biotin-low-density lipoprotein. A rapid new method for ligand blotting. Biochem. J. 229, 785-790. 

Fernandez LM, Lin ECK, Trejo A and McNamara DJ. 1992. Prickly pear (Opuntia sp.) pectin reverses low density lipoprotein receptor suppression induced by a hypercholesterolemic diet in Guinea Pigs. J Nutt 122 2330-2340. 

Salleh MN, Runnie I, Roach, PD, Mohamed S and Abeywardena MY. 2002. Inhibition of low-density lipoprotein oxidation and up-regulation of low-density lipoprotein receptor in HepG2 cells by tropical plant extracts. J Agric Food Chem 50(13) 3693-3697. 

The answer is a. (Katzung, p 590.) Bile acids are absorbed primarily in the ileum of the small intestine. Cholestyramine binds bile acids, preventing their reabsorption in the jejunum and ileum. Up to 10-fold greater excretion of bile acids occurs with the use of resins. The increased clearance leads to increased cholesterol turnover of bile acids. Low-density lipoprotein receptor upregulation results in increased uptake of LDL. This does not occur in homozygous familial hypercholesterolemia because of lack of functioning receptors. 

---