Multidrug permeases and subcellular cholesterol transport (original) (raw)
Brown, M. S. & Goldstein, J. L. A receptor mediated pathway for cholesterol homeostasis. Science232, 34–47 (1986). ArticleCASPubMed Google Scholar
Lange, Y. & Steck, T. L. The role of intracellular cholesterol transport in cholesterol homeostasis. Trends Cell Biol.6, 205–208 (1996). ArticleCASPubMed Google Scholar
Liscum, L. & Underwood, K. W. Intracellular cholesterol transport and compartmentation. J. Biol. Chem.270, 15443–15446 (1995). ArticleCASPubMed Google Scholar
Hampton, R., Dimster-Denk, D. & Rine, J. The biology of HMG-CoA reductase: the pros of contra-regulation. Trends Biochem. Sci.21, 140–145 (1996). ArticleCASPubMed Google Scholar
Roitelman, J., Olender, E. H., Bar-Nun, S., Dunn, W. A. Jr & Simoni, R. D. Immunological evidence for eight spans in the membrane domain of 3-hydroxy-3-methylglutaryl coenzyme A reductase: implications for enzyme degradation in the endoplasmic reticulum. J. Cell Biol.117, 959–973 (1992). ArticleCASPubMed Google Scholar
Gil, G., Faust, J. R., Chin, D. J., Goldstein, J. L. & Brown, M. S. Membrane-bound domain of HMG-CoA reductase is required for sterol-enhanced degradation of the enzyme. Cell41, 249–258 (1985). ArticleCASPubMed Google Scholar
Chang, C. C., Huh, H. Y., Cadigan, K. M. & Chang, T. Y. Molecular cloning and functional expression of human acyl-coenzyme A: cholesterol acyltransferase cDNA in mutant Chinese hamster ovary cells. J. Biol. Chem.268, 20747–20755 (1993). CASPubMed Google Scholar
Havel, R. J. & Kane, J. P. in The Metabolic and Molecular Bases of Inherited Disease (eds Scriver, C. R., Beaudet, A. L., Sly, W. S. & Valle, D.) 2705–2716 (McGraw–Hill, New York, 2001). Google Scholar
Dawson, G., Kruski, A. W. & Scanu, A. M. Distribution of glycosphingolipids in the serum lipoproteins of normal human subjects and patients with hypo- and hyperlipidemias. J. Lipid Res.17, 125–131 (1976). CASPubMed Google Scholar
Liscum, L. Compartmentation of cholesterol within the cell. Curr. Opin. Lipidol.5, 221–226 (1994). ArticleCASPubMed Google Scholar
Schroeder, F. et al. Recent advances in membrane cholesterol domain dynamics and intracellular cholesterol trafficking. Proc. Soc. Exp. Biol. Med.213, 150–177 (1996). ArticleCASPubMed Google Scholar
DeGrella, R. F. & Simoni, R. D. Intracellular transport of cholesterol to the plasma membrane. J. Biol. Chem.257, 14256–14262 (1982). CASPubMed Google Scholar
Urbani, L. & Simoni, R. D. Cholesterol and vesicular stomatitis virus G protein take separate routes from the endoplasmic reticulum to the plasma membrane. J. Biol. Chem.265, 1919–1923 (1990). CASPubMed Google Scholar
Kaplan, M. R. & Simoni, R. D. Transport of cholesterol from the endoplasmic reticulum to the plasma membrane. J. Cell Biol.101, 446–453 (1985). ArticleCASPubMed Google Scholar
Neufeld, E. B. et al. Intracellular trafficking of cholesterol monitored with a cyclodextrin. J. Biol. Chem.271, 21604–21613 (1996). ArticleCASPubMed Google Scholar
Spillane, D. M., Reagan, J. W. Jr, Kennedy, N. J., Scheidner, D. L. & Chang, T.-Y. Translocation of both lysosomal LDL-derived cholesterol and plasma membrane cholesterol to the endoplasmic reticulum for esterification may require common cellular factors involved in cholesterol egress from the acidic compartments (lysosomes/endosomes). Biochim. Biophys. Acta1254, 283–294 (1995). ArticlePubMed Google Scholar
Cruz, J. C., Sugii, S., Yu, C. & Chang, T. Y. Role of Niemann–Pick type C1 protein in intracellular trafficking of low density lipoprotein-derived cholesterol. J. Biol. Chem.275, 4013–4021 (2000).This work describes for the first time that LDL-derived cholesterol is rapidly transported to the plasma membrane, independently of the function of the NPC1 protein, which was previously thought to regulate such transport. ArticleCASPubMed Google Scholar
Lange, Y., Ye, J., Rigney, M. & Steck, T. Cholesterol movement in Niemann–Pick type C cells and in cells treated with amphiphiles. J. Biol. Chem.275, 17468–17475 (2000). ArticleCASPubMed Google Scholar
Liscum, L. Pharmacological inhibition of the intracellular transport of low-density lipoprotein-derived cholesterol in Chinese hamster ovary cells. Biochim. Biophys. Acta1045, 40–48 (1990). ArticleCASPubMed Google Scholar
Pentchev, P. G. et al. The Niemann–Pick C lesion and its relationship to the intracellular distribution and utilization of LDL cholesterol. Biochim. Biophys. Acta1225, 235–243 (1994). ArticleCASPubMed Google Scholar
Dupree, P., Parton, R. G., Raposo, G., Kurzchalia, T. V. & Simons, K. Caveolae and sorting in the _trans_-Golgi network of epithelial cells. EMBO J.12, 1597–1605 (1993). ArticleCASPubMedPubMed Central Google Scholar
Smart, E. J., Ying, Y.-S., Donzell, W. C. & Anderson, R. G. W. A role for caveolin in transport of cholesterol from endoplasmic reticulum to plasma membrane. J. Biol. Chem.271, 29427–29435 (1996). ArticleCASPubMed Google Scholar
Hannan, L. A. & Edidin, M. Traffic, polarity, and detergent solubility of a glycosylphosphatidylinositol-anchored protein after LDL-deprivation of MDCK cells. J. Cell Biol.133, 1265–1276 (1996). ArticleCASPubMed Google Scholar
Muller, G. et al. Redistribution of glycolipid raft domain components induces insulin-mimetic signaling in rat adipocytes. Mol. Cell. Biol.21, 4553–4567 (2001). ArticleCASPubMedPubMed Central Google Scholar
Bist, A., Fielding, P. E. & Fielding, C. J. Two sterol regulatory element-like sequences mediate up-regulation of caveolin gene transcription in response to low density lipoprotein free cholesterol. Proc. Natl Acad. Sci. USA94, 10693–10698 (1997). ArticleCASPubMedPubMed Central Google Scholar
Garver, W. S. et al. Increased expression of caveolin-1 in heterozygous Niemann–Pick type II human fibroblasts. Biochem. Biophys. Res. Commun.236, 189–193 (1997). ArticleCASPubMed Google Scholar
Garver, W. S. et al. Altered expression of caveolin-1 and increased cholesterol in detergent insoluble membrane fractions from liver in mice with Niemann–Pick disease type C. Biochim. Biophys. Acta1361, 272–280 (1997). ArticleCASPubMed Google Scholar
Pol, A. et al. A caveolin dominant-negative mutant associates with lipid bodies and induces intracellular cholesterol imbalance. J. Cell Biol.152, 1057–1070 (2001). ArticleCASPubMedPubMed Central Google Scholar
Fujimoto, T., Kogo, H., Ishiguro, K., Tauchi, K. & Nomura, R. Caveolin-2 is targeted to lipid droplets, a new 'membrane domain' in the cell. J. Cell Biol.152, 1079–1085 (2001).References29and30describe the discovery of new membrane domains in cells, and their association with caveolin. Great morphological studies and excellent time-lapse microscopy. ArticleCASPubMedPubMed Central Google Scholar
Simons, K. & Ikonen, E. Functional rafts in cell membranes. Nature387, 569–572 (1997).Proposes and demonstrates the existence of lipid rafts within the plasma membrane of mammalian cells. ArticleCASPubMed Google Scholar
Borst, P., Zelcer, N. & van Helvoort, A. ABC transporters in lipid transport. Biochim. Biophys. Acta1486, 128–144 (2000). ArticleCASPubMed Google Scholar
Schmitz, G., Kaminski, W. E. & Orso, E. ABC transporters in cellular lipid trafficking. Curr. Opin. Lipidol.11, 493–501 (2000).References32and33are excellent reviews on the involvement of ABC transporters in cellular lipid transport. ArticleCASPubMed Google Scholar
Dean, M., Hamon, Y. & Chimini, G. The human ATP-binding cassette (ABC) transporter superfamily. J. Lipid Res.42, 1007–1017 (2001). CASPubMed Google Scholar
Klein, I., Sarkadi, B. & Varadi, A. An inventory of the human ABC proteins. Biochim. Biophys. Acta1461, 237–262 (1999). ArticleCASPubMed Google Scholar
Nelissen, B., De Wachter, R. & Goffeau, A. Classification of all putative permeases and other membrane plurispanners of the major facilitator superfamily encoded by the complete genome of Saccharomyces cerevisiae. FEMS Microbiol. Rev.21, 113–134 (1997). ArticleCASPubMed Google Scholar
Rogers, B. et al. The pleiotropic drug ABC transporters from Saccharomyces cerevisiae. J. Mol. Microbiol. Biotechnol.3, 207–214 (2001). CASPubMed Google Scholar
Saier, M. H. Jr et al. Evolutionary origins of multidrug and drug-specific efflux pumps in bacteria. FASEB J.12, 265–274 (1998). ArticleCASPubMed Google Scholar
Van Bambeke, F., Balzi, E. & Tulkens, P. M. Antibiotic efflux pumps. Biochem. Pharmacol.60, 457–470 (2000). ArticleCASPubMed Google Scholar
Cabrita, M. A., Hobman, T. C., Hogue, D. L., King, K. M. & Cass, C. E. Mouse transporter protein, a membrane protein that regulates cellular multidrug resistance, is localized to lysosomes. Cancer Res.59, 4890–4897 (1999). CASPubMed Google Scholar
Paulsen, I. T., Brown, M. H. & Skurray, R. A. Proton-dependent multidrug efflux systems. Microbiol. Rev.60, 575–608 (1996). CASPubMedPubMed Central Google Scholar
Liu, Y. et al. A cDNA that suppresses MPP+ toxicity encodes a vesicular amine transporter. Cell70, 539–551 (1992). ArticleCASPubMed Google Scholar
Kaminski, W. E. et al. Identification of a novel human sterol-sensitive ATP-binding cassette transporter (ABCA7). Biochem. Biophys. Res. Commun.273, 532–538 (2000). ArticleCASPubMed Google Scholar
Bodzioch, M. et al. The gene encoding ATP-binding cassette transporter 1 is mutated in Tangier disease. Nature Genet.22, 347–351 (1999). ArticleCASPubMed Google Scholar
Brooks-Wilson, A. et al. Mutations in ABC1 in Tangier disease and familial high-density lipoprotein deficiency. Nature Genet.22, 336–345 (1999). ArticleCASPubMed Google Scholar
Rust, S. et al. Tangier disease is caused by mutations in the gene encoding ATP-binding cassette transporter 1. Nature Genet.22, 352–355 (1999).References45–47describe the molecular defect in Tangier disease, setting in motion an explosion in the field of ABC transporters and their involvement in lipid transport. ArticleCASPubMed Google Scholar
Mott, S. et al. Decreased cellular cholesterol efflux is a common cause of familial hypoalphalipoproteinemia: role of the ABCA1 gene mutations. Atherosclerosis152, 457–468 (2000). ArticleCASPubMed Google Scholar
Clee, S. M. et al. Age and residual cholesterol efflux affect HDL cholesterol levels and coronary artery disease in ABCA1 heterozygotes. J. Clin. Invest.106, 1263–1270 (2000). ArticleCASPubMedPubMed Central Google Scholar
Costet, P., Luo, Y., Wang, N. & Tall, A. R. Sterol-dependent transactivation of the ABC1 promoter by the liver X receptor/retinoid X receptor. J. Biol. Chem.275, 28240–28245 (2000). CASPubMed Google Scholar
Berge, K. E. et al. Accumulation of dietary cholesterol in sitosterolemia caused by mutations in adjacent ABC transporters. Science290, 1771–1775 (2000). ArticleCASPubMed Google Scholar
Walker, J. E., Saraste, M., Runswick, M. J. & Gay, N. J. Distantly related sequences in the α- and β-subunits of ATP synthase, myosin, kinases and other ATP-requiring enzymes and a common nucleotide binding fold. EMBO J.1, 945–951 (1982). ArticleCASPubMedPubMed Central Google Scholar
Wang, N., Silver, D. L., Costet, P. & Tall, A. R. Specific binding of ApoA-I, enhanced cholesterol efflux, and altered plasma membrane morphology in cells expressing ABC1. J. Biol. Chem.275, 33053–33058 (2000). ArticleCASPubMed Google Scholar
Mendez, A. J., Lin, G., Wade, D. P., Lawn, R. M. & Oram, J. F. Membrane lipid domains distinct from cholesterol/sphingomyelin-rich rafts are involved in the ABCA1-mediated lipid secretory pathway. J. Biol. Chem.276, 3158–3166 (2001). ArticleCASPubMed Google Scholar
Wang, N., Silver, D. L., Thiele, C. & Tall, A. R. ABCA1 functions as a cholesterol efflux regulatory protein. J. Biol. Chem.276, 23742–23747 (2001). ArticleCASPubMed Google Scholar
Vanier, M. T. et al. Type C Niemann–Pick disease: spectrum of phenotypic variation in disruption of intracellular LDL-drerived cholesterol processing. Biochim. Biophys. Acta1096, 328–337 (1991). ArticleCASPubMed Google Scholar
Patterson, M. C. et al. in The Metabolic and Molecular Bases of Inherited Disease (eds Scriver, C. R., Beaudet, A. L., Sly, W. S. & Valle, D.) 3611–3634 (McGraw–Hill, New York, 2001). Google Scholar
Pentchev, P. G. et al. Type C Niemann–Pick disease: a parallel loss of regulatory responses in both the uptake and esterification of low-density lipoprotein-derived cholesterol in cultured fibroblasts. J. Biol. Chem.261, 16775–16780 (1986). CASPubMed Google Scholar
Carstea, E. D. et al. Niemann–Pick C1 disease gene: homology to mediators of cholesterol homeostasis. Science277, 228–231 (1997). ArticleCASPubMed Google Scholar
Morris, J. A. et al. The genomic organization and polymorphism analysis of the human Niemann–Pick C1 gene. Biochem. Biophys. Res. Commun.261, 493–498 (1999). ArticleCASPubMed Google Scholar
Yamamoto, T. et al. NPC1 gene mutations in Japanese patients with Niemann–Pick disease type C. Hum. Genet.105, 10–16 (1999). CASPubMed Google Scholar
Millat, G. et al. Niemann–Pick C1 disease: the I1061T substitution is a frequent mutant allele in patients of Western European descent and correlates with a classic juvenile phenotype. Am. J. Hum. Genet.65, 1321–1329 (1999). ArticleCASPubMedPubMed Central Google Scholar
Greer, W. L. et al. Mutations in NPC1 highlight a conserved NPC1-specific cysteine-rich domain. Am. J. Hum. Genet.65, 1252–1260 (1999). ArticleCASPubMedPubMed Central Google Scholar
Greer, W. L. et al. The Nova Scotia (type D) form of NiemannPick disease is caused by a G3097→T transversion in NPC1. Am. J. Hum. Genet.63, 52–54 (1998). ArticleCASPubMedPubMed Central Google Scholar
Sun, X. et al. Niemann–Pick C variant detection by altered sphingolipid trafficking and correlation with mutations within a specific domain of NPC1. Am. J. Hum. Genet.68, 1361–1372 (2001). ArticleCASPubMedPubMed Central Google Scholar
Millat, G. et al. Niemann–Pick C1 disease: correlations between NPC1 mutations, levels of NPC1 protein, and phenotypes emphasize the functional significance of the putative sterol-sensing domain and of the cysteine-rich luminal loop. Am. J. Hum. Genet.68, 1373–1385 (2001). ArticleCASPubMedPubMed Central Google Scholar
Pentchev, P. G. et al. Group C Niemann–Pick disease: faulty regulation of low-density lipoprotein uptake and cholesterol storage in cultured fibroblasts. FASEB J.1, 40–45 (1987). ArticleCASPubMed Google Scholar
Liscum, L. & Faust, J. R. Low density lipoprotein (LDL)-mediated suppression of cholesterol synthesis and LDL uptake is defective in Niemann–Pick type C fibroblasts. J. Biol. Chem.262, 17002–17008 (1987). CASPubMed Google Scholar
Cadigan, K. M., Spillane, D. M. & Chang, T. Y. Isolation and characterization of Chinese hamster ovary cell mutants defective in intracellular low density lipoprotein-cholesterol trafficking. J. Cell Biol.110, 295–308 (1990). ArticleCASPubMed Google Scholar
Dahl, N. K., Reed, K. L., Daunais, M. A., Faust, J. R. & Liscum, L. Isolation and characterization of Chinese hamster ovary cells defective in the intracellular metabolism of low density lipoprotein-derived cholesterol. J. Biol. Chem.267, 4889–4896 (1992). CASPubMed Google Scholar
Davies, J. P., Chen, F. W. & Ioannou, Y. A. Transmembrane molecular pump activity of Niemann–Pick C1 protein. Science290, 2295–2298 (2000). ArticleCASPubMed Google Scholar
Marigo, V., Davey, R. A., Zuo, Y., Cunningham, J. M. & Tabin, C. J. Biochemical evidence that patched is the hedgehog receptor. Nature384, 176–179 (1996). ArticleCASPubMed Google Scholar
Stone, D. M. et al. The tumour suppressor gene patched encodes a candidate receptor for sonic hedgehog. Nature384, 129–133 (1996). ArticleCASPubMed Google Scholar
Fietz, M. J. et al. The hedgehog gene family in Drosophila and vertebrate development. Dev. Suppl. 43–51 (1994).
Lee, J. J. et al. Autoproteolysis in hedgehog protein biogenesis. Science266, 1528–1537 (1994). ArticleCASPubMed Google Scholar
Hua, X., Nohturfft, A., Goldstein, J. L. & Brown, M. S. Sterol resistance in CHO cells traced to point mutation in SREBP cleavage-activating protein. Cell87, 415–426 (1996). ArticleCASPubMed Google Scholar
Davies, J. P., Levy, B. & Ioannou, Y. A. Evidence for a Niemann–Pick C (NPC) gene family: identification and characterization of NPC1L1. Genomics65, 137–145 (2000). ArticleCASPubMed Google Scholar
Neufeld, E. B. et al. The Niemann–Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo. J. Biol. Chem.274, 9627–9635 (1999). ArticleCASPubMed Google Scholar
Patel, S. C. et al. Localization of Niemann–Pick C1 protein in astrocytes: implications for neuronal degeneration in Niemann–Pick type C disease. Proc. Natl Acad. Sci. USA96, 1657–1662 (1999). ArticleCASPubMedPubMed Central Google Scholar
Higgins, M. E., Davies, J. P., Chen, F. W. & Ioannou, Y. A. Niemann–Pick C1 is a late endosome-resident protein that transiently associates with lysosomes and the _trans_-Golgi network. Mol. Genet. Metab.68, 1–13 (1999). ArticleCASPubMed Google Scholar
Kobayashi, T. et al. Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol transport. Nature Cell Biol.1, 113–118 (1999).The first report to establish the late endosome as the cholesterol storage compartment inNPC−/−cells and the role of lysobisphosphatidic acid in regulating cholesterol transport. ArticleCASPubMed Google Scholar
Puri, V. et al. Cholesterol modulates membrane traffic along the endocytic pathway in sphingolipid-storage diseases. Nature Cell Biol.1, 386–388 (1999).An excellent paper describing the involvement of cholesterol in the regulation of membrane transport. ArticleCASPubMed Google Scholar
Ko, D. C., Gordon, M. D., Jin, J. Y. & Scott, M. P. Dynamic movements of organelles containing Niemann–Pick C1 protein: NPC1 involvement in late endocytic events. Mol. Biol. Cell12, 601–614 (2001). ArticleCASPubMedPubMed Central Google Scholar
Zhang, M. et al. Cessation of rapid late endosomal tubulovesicular trafficking in Niemann–Pick type C1 disease. Proc. Natl Acad. Sci. USA98, 4466–4471 (2001). ArticleCASPubMedPubMed Central Google Scholar
Zhang, M. et al. Sterol-modulated glycolipid sorting occurs in Niemann–Pick C1 late endosomes. J. Biol. Chem.276, 3417–3425 (2001). ArticleCASPubMed Google Scholar
Millard, E. E., Srivastava, K., Traub, L. M., Schaffer, J. E. & Ory, D. S. Niemann–Pick type C1 (NPC1) overexpression alters cellular cholesterol homeostasis. J. Biol. Chem.275, 38445–38451 (2000). ArticleCASPubMed Google Scholar
Fukuda, M., Viitala, J., Matteson, J. & Carlsson, S. R. Cloning of cDNAs encoding human lysosomal membrane glycoproteins, h-lamp-1 and h-lamp-2. J. Biol. Chem.262, 18920–18928 (1988). Google Scholar
Tseng, T.-T. et al. The RND permease superfamily: an ancient, ubiquitous and diverse family that includes human disease and development proteins. J. Mol. Microbiol. Biotechnol.1, 107–125 (1999). CASPubMed Google Scholar
Kirchhoff, C., Osterhoff, C. & Young, L. Molecular cloning and characterization of HE1, a major secretory protein of the human epididymis. Biol. Reprod.54, 847–856 (1996). ArticleCASPubMed Google Scholar
Naureckiene, S. et al. Identification of HE1 as the second gene of Niemann–Pick C disease. Science290, 2298–2301 (2000).Elucidation of the molecular defect in NPC type 2 disease. ArticleCASPubMed Google Scholar
Okamura, N. et al. A porcine homolog of the major secretory protein of human epididymis, HE1, specifically binds cholesterol. Biochim. Biophys. Acta1438, 377–387 (1999). ArticleCASPubMed Google Scholar
Nakamura, H. & Ohtsubo, K. Ultrastructure appearance of atherosclerosis in human and experimentally-induced animal models. Electron Microsc. Rev.5, 129–170 (1992). ArticleCASPubMed Google Scholar
Tangirala, R. K. et al. Formation of cholesterol monohydrate crystals in macrophage-derived foam cells. J. Lipid Res.35, 93–104 (1994). CASPubMed Google Scholar
Shio, H., Fowler, S., Bhuvaneswaran, C. & Morris, M. D. Lysosome lipid storage disorder in NCTR-BALB/c mice. II. Morphologic and cytochemical studies. Am. J. Pathol.108, 150–159 (1982). CASPubMedPubMed Central Google Scholar
Tomasetto, C. et al. Identification of four novel human genes amplified and overexpressed in breast carcinoma and localized to the q11–q21.3 region of chromosome 17. Genomics28, 367–376 (1995). ArticleCASPubMed Google Scholar
Moog-Lutz, C. et al. MLN64 exhibits homology with the steroidogenic acute regulatory protein (STAR) and is over-expressed in human breast carcinomas. Int. J. Cancer71, 183–191 (1997). ArticleCASPubMed Google Scholar
Watari, H. et al. MLN64 contains a domain with homology to the steroidogenic acute regulatory protein (StAR) that stimulates steroidogenesis. Proc. Natl Acad. Sci. USA94, 8462–8467 (1997). ArticleCASPubMedPubMed Central Google Scholar
Tsujishita, Y. & Hurley, J. H. Structure and lipid transport mechanism of a StAR-related domain. Nature Struct. Biol.7, 408–414 (2000). ArticleCASPubMed Google Scholar
Alpy, F. et al. The steroidogenic acute regulatory protein homolog MLN64, a late endosomal cholesterol-binding protein. J. Biol. Chem.276, 4261–4269 (2001). ArticleCASPubMed Google Scholar
Ericsson, J., Jackson, S. M., Lee, B. C. & Edwards, P. A. Sterol regulatory binding element protein binds to cis element in the promoter of the farnesyl diphosphate gene. Proc. Natl Acad. Sci. USA93, 945–950 (1996). ArticleCASPubMedPubMed Central Google Scholar
Ericsson, J., Usheva, A. & Edwards, P. A. YY1 is a negative regulator of transcription of three sterol regulatory element-binding protein-responsive genes. J. Biol. Chem.274, 14508–14513 (1999). ArticleCASPubMed Google Scholar
Shrivastava, A. & Calame, K. An analysis of genes regulated by the multi-functional transcriptional regulator Yin Yang-1. Nucleic Acids Res.22, 5151–5155 (1994). ArticleCASPubMedPubMed Central Google Scholar
Cooper, M. K., Porter, J. A., Young, K. E. & Beachy, P. A. Teratogen-mediated inhibition of target tissue response to Shh signaling. Science280, 1603–1607 (1998). ArticleCASPubMed Google Scholar
Porter, J. P., Young, K. E. & Beachy, P. A. Cholesterol modification of hedgehog signaling protein in animal development. Science274, 255–259 (1996).An excellent paper, demonstrating the autoproteolysis and cholesterol modification of Hedghog protein. ArticleCASPubMed Google Scholar
Alcedo, J., Ayzenzon, M., Von Ohlen, T., Noll, M. & Hooper, J. E. The Drosophila smoothened gene encodes a seven-pass membrane protein, a putative receptor for the hedgehog signal. Cell86, 221–232 (1996). ArticleCASPubMed Google Scholar
Hahn, H., Wojnowski, L., Miller, G. & Zimmer, A. The patched signaling pathway in tumorigenesis and development: lessons from animal models. J. Mol. Med.77, 459–468 (1999). ArticleCASPubMed Google Scholar
Johnson, R. L. et al. Human homolog of patched, a candidate gene for the basal cell nevus syndrome. Science272, 1668–1671 (1996). ArticleCASPubMed Google Scholar
Reifenberger, J. et al. Missense mutations in SMOH in sporadic basal cell carcinomas of the skin and primitive neuroectodermal tumors of the central nervous system. Cancer Res.58, 1798–1803 (1998). CASPubMed Google Scholar
Raffel, C. et al. Sporadic medulloblastomas contain PTCH mutations. Cancer Res.57, 842–845 (1997). CASPubMed Google Scholar
Martin, V., Carrillo, G., Torroja, C. & Guerrero, I. The sterol-sensing domain of Patched protein seems to control Smoothened activity through Patched vesicular trafficking. Curr. Biol.11, 601–607 (2001). ArticleCASPubMed Google Scholar
Strutt, H. et al. Mutations in the sterol-sensing domain of Patched suggest a role for vesicular trafficking in Smoothened regulation. Curr. Biol.11, 608–613 (2001). ArticleCASPubMed Google Scholar
Groener, J. E., Bax, W. & Poorthuis, B. J. Metabolic fate of oleic acid derived from lysosomal degradation of cholesteryl oleate in human fibroblasts. J. Lipid Res.37, 2271–2279 (1996). CASPubMed Google Scholar
Berk, P. D. & Stump, D. D. Mechanisms of cellular uptake of long chain free fatty acids. Mol. Cell Biochem.192, 17–31 (1999). ArticleCASPubMed Google Scholar
Dutta-Roy, A. K. Cellular uptake of long-chain fatty acids: role of membrane-associated fatty-acid-binding/transport proteins. Cell. Mol. Life Sci.57, 1360–1372 (2000). ArticleCASPubMed Google Scholar
Kennedy, M. W. & Beauchamp, J. Sticky-finger interaction sites on cytosolic lipid-binding proteins? Cell. Mol. Life Sci.57, 1379–1387 (2000). ArticleCASPubMed Google Scholar
Stewart, J. M. The cytoplasmic fatty-acid-binding proteins: thirty years and counting. Cell. Mol. Life Sci.57, 1345–1359 (2000). ArticleCASPubMed Google Scholar
Ou, J. et al. Unsaturated fatty acids inhibit transcription of the sterol regulatory element-binding protein-1c (SREBP-1c) gene by antagonizing ligand-dependent activation of the LXR. Proc. Natl Acad. Sci. USA98, 6027–6032 (2001). ArticleCASPubMedPubMed Central Google Scholar
Xu, J., Teran-Garcia, M., Park, J. H., Nakamura, M. T. & Clarke, S. D. Polyunsaturated fatty acids suppress hepatic sterol regulatory element- binding protein-1 expression by accelerating transcript decay. J. Biol. Chem.276, 9800–9807 (2001). ArticleCASPubMed Google Scholar
Hannah, V. C., Ou, J., Luong, A., Goldstein, J. L. & Brown, M. S. Unsaturated fatty acids down-regulate SREPB isoforms 1a and 1c by two mechanisms in HEK-293 cells. J. Biol. Chem.276, 4365–4372 (2001). ArticleCASPubMed Google Scholar
Pai, J. T., Guryev, O., Brown, M. S. & Goldstein, J. L. Differential stimulation of cholesterol and unsaturated fatty acid biosynthesis in cells expressing individual nuclear sterol regulatory element-binding proteins. J. Biol. Chem.273, 26138–26148 (1998). ArticleCASPubMed Google Scholar
Schoer, J. K. et al. Lysosomal membrane cholesterol dynamics. Biochemistry39, 7662–7677 (2000).A great study that demonstrates the requirement of processes extrinsic to the lysosomal membrane for efficient cholesterol exit from this compartment. ArticleCASPubMed Google Scholar
Zervas, M., Dobrenis, K. & Walkley, S. U. Neurons in Niemann–Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations. J. Neuropathol. Exp. Neurol.60, 49–64 (2001). ArticleCASPubMed Google Scholar
Harzer, K. & Kustermann-Kuhn, B. Quantified increases of cholesterol, total lipid and globotriaosylceramide in filipin-positive Niemann–Pick type C fibroblasts. Clin. Chim. Acta305, 65–73 (2001). ArticleCASPubMed Google Scholar
Yamazaki, T., Chang, T. Y., Haass, C. & Ihara, Y. Accumulation and aggregation of amyloid beta-protein in late endosomes of Niemann–Pick type C cells. J. Biol. Chem.276, 4454–4460 (2001). ArticleCASPubMed Google Scholar
Simons, K. & Gruenberg, J. Jamming the endosomal system: lipid rafts and lysosomal storage diseases. Trends Cell Biol.10, 459–462 (2000). ArticleCASPubMed Google Scholar