Cholesterol biosynthesis and regulation: Role of peroxisomes

Advances in Experimental Medicine and Biology

Volumn 544, Issue , 2003, Pages 315-327

  Kovacs, Werner J ^a   Krisans, Skaidrite ^a  

^a SAN DIEGO STATE UNIVERSITY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ACETYL COENZYME A ACETYLTRANSFERASE; CHOLESTEROL; GERANYLTRANSFERASE; HYDROXYMETHYLGLUTARYL COENZYME A REDUCTASE; HYDROXYMETHYLGLUTARYL COENZYME A SYNTHASE; ISOPENTENYL DIPHOSPHATE DELTA ISOMERASE; MEVALONATE DIPHOSPHATE DECARBOXYLASE; MEVALONATE KINASE; PHOSPHOMEVALONATE KINASE; PHOSPHOTRANSFERASE; UNCLASSIFIED DRUG;

AMINO ACID SEQUENCE; BIOGENESIS; CARBOXY TERMINAL SEQUENCE; CATALYSIS; CATALYST; CELLULAR DISTRIBUTION; CHOLESTEROL BLOOD LEVEL; CHOLESTEROL SYNTHESIS; CONFERENCE PAPER; CYTOSOL; DISORDERS OF PEROXISOMAL FUNCTIONS; ENZYME ACTIVITY; FIBROBLAST; GENOME; HOMEOSTASIS; HUMAN; KNOCKOUT MOUSE; NONHUMAN; PEROXISOME; PRIORITY JOURNAL; PROTEIN TARGETING; SIGNAL TRANSDUCTION;

EID: 1542329066     PISSN: 00652598     EISSN: None     Source Type: Book Series    
DOI: 10.1007/978-1-4419-9072-3_41     Document Type: Conference Paper

References (55)

1. Aboushadi, N., and Krisans, S.K., 1998, Analysis of isoprenoid biosynthesis in peroxisomal-deficient Pex2 CHO cell lines. J. Lipid Res. 39: 1781-1791.
2. Ashby, M.N., and Edwards, P.A., 1989, Identification and regulation of a rat liver cDNA encoding farnesyl pyrophosphate synthetase. J. Biol. Chem. 264: 635-640.
3. Ayte, J., Gil-Gomez, G., Haro, D., Marrero, P.F., and Hegardt, F.G., 1990, Rat mitochondrial and cytosolic 3-hydroxy-3-methylglutaryl-CoA synthases are encoded by two different genes. Proc. Natl. Acad. Sci. USA 87: 3874-3878.
4. Baes, M., Gressens, P., Baumgart, E., Carmeliet, P., Casteels, M., Fransen, M., Evrard, P., Fahimi, D., Declercq, P.E., Collen, D., van Veldhoven, P.P., and Mannaerts, G.P., 1997, A mouse model for Zellweger syndrome. Nat. Genet. 17: 49-57.
5. Biardi, L., and Krisans, S.K., 1996, Compartmentalization of cholesterol biosynthesis. Conversion of mevalonate to farnesyl diphosphate occurs in the peroxisomes. J. Biol. Chem. 271: 1784-1788.
6. Biardi, L., Sreedhar, A., Zokaei, A., Vartak, N.B., Bozeat, R.L., Shackelford, J.E., Keller, G.A., and Krisans, S.K., 1994, Mevalonate kinase is predominantly localized in peroxisomes and is defective in patients with peroxisome deficiency disorders. J. Biol. Chem. 269: 1197-1205.
7. Bjorkman, J., Tonks, I., Maxwell, M.A., Paterson, C., Kay, G.F., and Crane, D.I., 2002, Conditional inactivation of the peroxisome biogenesis Pex13 gene by Cre-loxP excision. Genesis 32: 179-180.
8. Breitling, R., and Krisans, S.K., 2002, A second gene for peroxisomal HMG-CoA reductase? A genomic reassessment. J. Lipid Res. 43: 2031-2036.
9. Cohen, L.H., Griffioen, M., van Roermund, C.W., and Wanders, R.J., 1992, Subcellular localization of squalene synthase in human hepatoma cell line HepG2. Biochim. Biophys. Acta 1126: 114-118.
10. Collins, J.C., Keyserman, F., Rumsey, S.C., and Deckelbaum, R.J., 1993, Peroxisomal disease patients have hypocholesterolemia and abnormal cholesterol synthesis and LDL receptor regulation in vitro. American Heart Association (Abstract).
11. Dammai, V., and Subramani, S., 2001, The human peroxisomal targeting signal receptor, Pex5p, is translocated into the peroxisomal matrix and recycled to the cytosol. Cell 105: 187-196.
12. Elgersma, Y., Vos, A., van den Berg, M., van Roermund C.W.T., van ser Sluijs, P., Distel, B., and Tabak, H.F., 1996, Analysis of the carboxyl-terminal peroxisomal targeting signal 1 in a homologous context in Saccharomyces cerevisiae. J. Biol. Chem. 271: 26375-26382.
13. Engfelt, W.H., Shackelford, J.E., Aboushadi, N., Jessani, N., Masuda, K., Paton, V.G., Keller, G.A., and Krisans, S.K., 1997, Characterization of UT2 cells. The induction of peroxisomal 3-hydroxy-3-methylglutaryl-coenzyme A reductase. J. Biol. Chem. 272: 24579-24587.
14. Faust, P.L., and Hatten, M.E., 1997, Targeted deletion of the PEX2 peroxisome assembly gene in mice provides a model for Zellweger syndrome, a human neuronal migration disorder. J. Cell Biol. 139: 1293-1305.
15. Faust, P.L., Su, H., Moser, A., and Moser, H.W., 2001, The peroxisome deficient PEX2 mouse. Pathologic and biochemical correlates of lipid dysfunction. J. Mol. Neurosci. 16: 289-297.
16. Flynn, C.R., Mullen, R.T., and Trelease, R.N., 1998, Mutational analyses of a type 2 peroxisomal targeting signal that is capable of directing oligomeric protein import into tobacco BY-2 glyoxysomes. Plant J. 16: 709-720.
17. Fukao, T., Yamaguchi, S., Kano, M., Orii, T., Fujiki, Y., Osumi, T., and Hashimoto, T., 1990, Molecular cloning and sequence of the complementary DNA encoding human mitochondrial acetoacetyl-coenzyme A thiolase and study of the variant enzymes in cultured fibroblasts from patients with 3-ketothiolase deficiency. J. Clin. Invest. 86: 2086-2092.
18. Gietl, C., Faber, K.N., van der Klei, I.J., and Veenhuis, M., 1994, Mutational analysis of the N-terminal topogenic signal of watermelon glyoxysomal malate dehydrogenase using the heterologous host Hansenula polymorpha. Proc. Natl. Acad. Sci. USA 91: 3151-3155.
19. Gould, S.J., and Valle, D., 2000, Peroxisome biogenesis disorders: genetics and cell biology. Trends Genet. 16: 340-345.
20. Gould, S.J., Keller, G.A., and Subramani, S., 1987, Identification of a peroxisomal targeting signal at the carboxy terminus of firefly luciferase. J Cell Biol. 105: 2923-2931.
21. Hodge, V.J., Gould, S.J., Subramani, S., Moser, H.W., and Krisans, S.K., 1991, Normal cholesterol synthesis in human cells requires functional peroxisomes. Biochem. Biophys. Res. Commun. 181: 537-541.
22. Hogenboom, S., Romeijn, G.J., Houten, S.M., Baes, M., Wanders, R.J.A., and Waterham, H.R., 2002, Absence of functional peroxisomes does not lead to deficiency of enzymes involved in cholesterol biosynthesis. J. Lipid Res. 43: 90-98.
23. Horton, J.D., Goldstein, J.L., and Brown, M.S., 2002, SREBPs: activators of the complete program of cholesterol and fatty acid synthesis in the liver. J. Clin. Invest. 109: 1125-1131.
24. Hovik, R., Brodal, B., Bartlett, K., and Osmundsen, H., 1991, Metabolism of acetyl-CoA by isolated peroxisomal fractions: formation of acetate and acetoacetyl-CoA. J. Lipid Res. 32: 993-999.
25. Keller, G.A., Barton, M.C., Shapiro, D.J., and Singer, S.J., 1985, 3-Hydroxy-3-methylglutaryl-coenzyme A reductase is present in peroxisomes in normal rat liver cells. Proc. Natl. Acad. Sci. USA 82: 770-774.
26. Keller, G.A., Pazirandeh, M., and Krisans, S.K., 1986, 3-Hydroxy-3-methylglutaryl coenzyme A reductase localization in rat liver peroxisomes and microsomes of control and cholestyramine-treated animals: quantitative biochemical and immunoelectron microscopical analyses. J. Cell Biol. 103: 875-886.
27. Kovacs, W.J., Faust, P.L., Keller, G.Aa, and Krisans, S.K., 2001, Purification of brain peroxisomes and localization of 3-hydroxy-3- methylglutaryl coenzyme A reductase. Eur. J. Biochem. 268: 4850-4859.
28. Kovacs, W.J., Olivier, L.M., and Krisans, S.K., 2002, Central role of peroxisomes in isoprenoid biosynthesis. Prog. Lipid Res. 41: 369-391.
29. Krisans, S.K., 1996, Cell compartmentalization of cholesterol biosynthesis. Ann. N.Y. Acad. Sci. 804: 142-164.
30. Krisans, S.K., Ericsson, J., Edwards, P.A., and Keller, G.A., 1994, Farnesyl-diphosphate synthase is localized in peroxisomes. J. Biol. Chem. 269: 14165-14169.
31. Krisans, S.K., Rusnak, N., Keller, G.A., and Edwards, P.A., 1988, Localization of 3-hydroxy-3-methyl-glutaryl-coenzyme A synthase in rat liver peroxisomes. J. Cell Biol. 107: 122 (Abstract).
32. Lazarow, P.B., and Moser, H.W., 1989. In The metabolic basis of inherited disease (C.R. Scriver, A.L. Beaudet, W.S. Sly, and D. Valle, eds), McGraw-Hill, New York, pp. 1479-1509.
33. Lee, M.S., Mullen, R.T., and Trelease, R.N., 1997, Oilseed isocitrate lyases lacking their essential type 1 peroxisomal targeting signal are piggybacked to glyoxysomes. Plant Cell 9: 185-197.
34. Loewen, C.J.R., and Levine, T.P., 2002, Cholesterol homeostasis: Not until the SCAP lady INSIGs. Curr. Biol. 12: R779-R781.
35. Malle, E., Oettl, K., Sattler, W., Hoefler, G., and Kostner, G.M., 1995, Cholesterol biosynthesis in dermal fibroblasts from patients with metabolic disorders of peroxisomal origin. Eur. J. Clin. Invest. 25: 59-67.
36. Mandel, H., Getsis, M., Rosenblat, M., Berant, M., and Aviram, M., 1995, Reduced cellular cholesterol content in peroxisome-deficient fibroblasts is associated with impaired uptake of the patients' low density lipoprotein and with reduced cholesterol synthesis. J. Lipid Res. 36: 1385-1391.
37. McNew, J.A., and Goodman, J.M., 1994, An oligomeric protein is imported into peroxisomes in vivo. J. Cell Biol. 127: 1245-1257.
38. Oettl, K., Malle, E., Grillhofer, H., Sattler, W., and Kostner, G.M., 1996, Cholesterol metabolism in cells with different peroxisomal defects. Clin. Chim. Acta 251: 131-143.
39. Olivier, L.M., Chambliss, K.L., Gibson, K.M., and Krisans, S.K., 1999, Characterization of phosphomevalonate kinase: chromosomal localization, regulation, and subcellular targeting. J. Lipid Res. 40: 672-679.
40. Olivier, L.M., Kovacs, W., Masuda, K., Keller, G.A., and Krisans, S.K., 2000, Identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes: AA-CoA thiolase, HMG-CoA synthase, MPPD, and FPP synthase. J. Lipid Res. 41: 1921-1935.
41. Paton, V.G., Shackelford, J.E., and Krisans, S.K., 1997, Cloning and subcellular localization of hamster and rat isopentenyl diphosphate dimethylallyl diphosphate isomerase. A PTS1 motif targets the enzyme to peroxisomes. J. Biol. Chem. 272: 18945-18950.
42. Slawecki, M.L., Dodt, G., Steinberg, S., Moser, A.B., Moser, H.W., and Gould, S.J., 1995, Identification of three distinct peroxisomal protein import defects in patients with peroxisome biogenesis disorders. J. Cell Sci. 108: 1817-1829.
43. Song, X.Q., Fukao, T., Yamaguchi, S., Miyazawa, S., Hashimoto, T., and Orii, T., 1994, Molecular cloning and nucleotide sequence of complementary DNA for human hepatic cytosolic acetoacetyl-coenzyme A thiolase. Biochem. Biophys. Res. Commun. 201: 478-485.
44. Stamellos, K.D., Shackelford, J.E., Shechter, I., Jiang, G., Conrad, D., Keller, G.A., and Krisans, S.K., 1993, Subcellular localization of squalene synthase in rat hepatic cells. Biochemical and immunochemical evidence. J. Biol. Chem. 268: 12825-12836.
45. Stamellos, K.D., Shackelford, J.E., Tanaka, R.D., and Krisans, S.K., 1992. Mevalonate kinase is localized in rat liver peroxisomes. J. Biol. Chem. 267: 5560-5568.
46. Swinkles, B.W., Gould, S.J., Bodnar, A.G., Rachubinski, R.A., and Subramani, S., 1991, A novel, cleavable peroxisomal targeting signal at the amino-terminus of the rat 3-ketoacyl-CoA thiolase. EMBO J. 10: 3255-3262.
47. Thompson, S.L., and Krisans, S.K., 1990, Rat liver peroxisomes catalyze the initial step in cholesterol synthesis: the condensation of acetyl-CoA units into acetoacetyl-CoA. J. Biol. Chem. 25: 5731-5735.
48. Tsukamoto, T., Hata, S., Yokota, S., Miura, S., Fujiki, Y., Hijikata, M., Miyazawa, S., Hashimoto, T., and Osumi, T., 1994, Characterization of the signal peptide at the amino terminus of the rat peroxisomal 3-ketoacyl-CoA thiolase precursor. J. Biol. Chem. 269: 6001-6010.
49. Tsukamoto, T., Miura, S., and Fujiki, Y., 1991, Restoration by a 35K membrane protein of peroxisome assembly in a peroxisome-deficient mammalian cell mutant. Nature 350: 77-81.
50. Van Heusden, G.P., van Beckhoven, J.R., Thieringer, R., Raetz, C.R., and Wirtz, K.W., 1992, Increased cholesterol synthesis in Chinese hamster ovary cells deficient in peroxisomes. Biochim. Biophys. Acta 1126: 81-87.
51. Vanhorebeek, I., Baes, M., and Declercq, P.E., 2001, Isoprenoid biosynthesis is not compromised in a Zellweger syndrome mouse model. Biochim. Biophys. Acta 1532: 28-36.
52. Walton, P.A., Hill, P.E., and Subramani, S., 1995, Import of stably folded proteins into peroxisomes. Mol. Biol. Cell 6: 675-683.
53. Wanders, R.J.A., and Romeijn, G.J., 1996, Cholesterol biosynthesis in Zellweger syndrome: Normal activity of mevalonate kinase, mevalonate-5- pyrophosphate decarboxylase and IPP-isomerase in patients' fibroblasts but deficient mevalonate kinase activity in liver. J. Inher. Metab. Dis. 19: 193-196.
54. Wanders, R.J.A., and Romeijn, G.J., 1998, Differential deficiency of mevalonate kinase and phosphomevalonate kinase in patients with distinct defects in peroxisome biogenesis: evidence for a major role of peroxisomes in cholesterol biosynthesis. Biochem. Biophys. Res. Commun. 247: 663-667.
55. Wilkin, D.J., Kutsunai, S.Y., and Edwards, P.A., 1990, Isolation and sequence of the human farnesyl pyrophosphate synthetase cDNA. Coordinate regulation of the mRNAs for farnesyl pyrophosphate synthetase, 3-hydroxy-3-methylglutaryl coenzyme A reductase, and 3-hydroxy-3-methylglutaryl coenzyme A synthase by phorbol ester. J. Biol. Chem. 265: 4607-4614.