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Disease relevance of Peroxisomes


High impact information on Peroxisomes


Chemical compound and disease context of Peroxisomes


Biological context of Peroxisomes


Anatomical context of Peroxisomes


Associations of Peroxisomes with chemical compounds

  • We now demonstrate that ultraspiracle (usp), a Drosophila RXR homolog, can substitute for RXR in stimulating the DNA binding of receptors for retinoic acid, T3, vitamin D, and peroxisome proliferator activators [26].
  • These results support prior studies emphasizing the role of peroxisomes and the acyl DHAP pathway in cellular ether lipid synthesis, establish Zellweger syndrome cells as valuable for elucidating peroxisomal functions, and provide prenatal and postnatal diagnostic assays as well as potential therapeutic strategies for Zellweger syndrome [2].
  • Peroxisome proliferator-activated receptors (PPARs) are key players in lipid and glucose metabolism and are implicated in metabolic disorders predisposing to atherosclerosis, such as dyslipidaemia and diabetes [27].
  • Activation of a member of the steroid hormone receptor superfamily by peroxisome proliferators [28].
  • Amphipathic carboxylates such as clofibric acid have been used in man as hypolipidaemic agents and in rodents they stimulate the proliferation of peroxisomes [17].

Gene context of Peroxisomes

  • Phenotypic analysis of PEX1-deficient cells revealed severe defects in peroxisomal matrix protein import and destabilization of PEX5, the receptor for the type-1 peroxisomal targetting signal, even though peroxisomes were present in these cells and capable of importing peroxisomal membrane proteins [10].
  • PEX7, a candidate gene for RCDP identified in yeast, encodes the receptor for peroxisomal matrix proteins with the type-2 peroxisome targeting signal (PTS2) [29].
  • PEX12 encodes an integral membrane protein of peroxisomes [30].
  • The Pas2 protein essential for peroxisome biogenesis is related to ubiquitin-conjugating enzymes [31].
  • When the mutant is transfected with a good PEX3 gene, these protoperoxisomes acquire additional membrane peroxins and then import the matrix enzymes to reform peroxisomes [19].

Analytical, diagnostic and therapeutic context of Peroxisomes


  1. Identification of PAHX, a Refsum disease gene. Mihalik, S.J., Morrell, J.C., Kim, D., Sacksteder, K.A., Watkins, P.A., Gould, S.J. Nat. Genet. (1997) [Pubmed]
  2. Deficiency of enzymes catalyzing the biosynthesis of glycerol-ether lipids in Zellweger syndrome. A new category of metabolic disease involving the absence of peroxisomes. Datta, N.S., Wilson, G.N., Hajra, A.K. N. Engl. J. Med. (1984) [Pubmed]
  3. Muscle-specific overexpression of lipoprotein lipase causes a severe myopathy characterized by proliferation of mitochondria and peroxisomes in transgenic mice. Levak-Frank, S., Radner, H., Walsh, A., Stollberger, R., Knipping, G., Hoefler, G., Sattler, W., Weinstock, P.H., Breslow, J.L., Zechner, R. J. Clin. Invest. (1995) [Pubmed]
  4. Suppression of liver cell apoptosis in vitro by the non-genotoxic hepatocarcinogen and peroxisome proliferator nafenopin. Bayly, A.C., Roberts, R.A., Dive, C. J. Cell Biol. (1994) [Pubmed]
  5. Mouse steroid receptor coactivator-1 is not essential for peroxisome proliferator-activated receptor alpha-regulated gene expression. Qi, C., Zhu, Y., Pan, J., Yeldandi, A.V., Rao, M.S., Maeda, N., Subbarao, V., Pulikuri, S., Hashimoto, T., Reddy, J.K. Proc. Natl. Acad. Sci. U.S.A. (1999) [Pubmed]
  6. Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis. Selverstone Valentine, J., Doucette, P.A., Zittin Potter, S. Annu. Rev. Biochem. (2005) [Pubmed]
  7. Carnitine--metabolism and functions. Bremer, J. Physiol. Rev. (1983) [Pubmed]
  8. Contribution of the endoplasmic reticulum to peroxisome formation. Hoepfner, D., Schildknegt, D., Braakman, I., Philippsen, P., Tabak, H.F. Cell (2005) [Pubmed]
  9. The human peroxisomal targeting signal receptor, Pex5p, is translocated into the peroxisomal matrix and recycled to the cytosol. Dammai, V., Subramani, S. Cell (2001) [Pubmed]
  10. Mutations in PEX1 are the most common cause of peroxisome biogenesis disorders. Reuber, B.E., Germain-Lee, E., Collins, C.S., Morrell, J.C., Ameritunga, R., Moser, H.W., Valle, D., Gould, S.J. Nat. Genet. (1997) [Pubmed]
  11. Lignoceric acid is oxidized in the peroxisome: implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy. Singh, I., Moser, A.E., Goldfischer, S., Moser, H.W. Proc. Natl. Acad. Sci. U.S.A. (1984) [Pubmed]
  12. Abnormality in catalase import into peroxisomes leads to severe neurological disorder. Sheikh, F.G., Pahan, K., Khan, M., Barbosa, E., Singh, I. Proc. Natl. Acad. Sci. U.S.A. (1998) [Pubmed]
  13. Inhibitors of the arachidonic acid pathway and peroxisome proliferator-activated receptor ligands have superadditive effects on lung cancer growth inhibition. Avis, I., Martínez, A., Tauler, J., Zudaire, E., Mayburd, A., Abu-Ghazaleh, R., Ondrey, F., Mulshine, J.L. Cancer Res. (2005) [Pubmed]
  14. Changes in peroxisomes in preneoplastic liver and hepatoma of mice induced by alpha-benzene hexachloride. Tsukada, H., Gotoh, M., Mochizuki, Y., Furukawa, K. Cancer Res. (1979) [Pubmed]
  15. Induction of peroxisomal fatty acid beta-oxidation and liver fatty acid-binding protein by peroxisome proliferators. Mediation via the cytochrome P-450IVA1 omega-hydroxylase pathway. Kaikaus, R.M., Chan, W.K., Lysenko, N., Ray, R., Ortiz de Montellano, P.R., Bass, N.M. J. Biol. Chem. (1993) [Pubmed]
  16. Mutations in the 70K peroxisomal membrane protein gene in Zellweger syndrome. Gärtner, J., Moser, H., Valle, D. Nat. Genet. (1992) [Pubmed]
  17. Convergence of 9-cis retinoic acid and peroxisome proliferator signalling pathways through heterodimer formation of their receptors. Kliewer, S.A., Umesono, K., Noonan, D.J., Heyman, R.A., Evans, R.M. Nature (1992) [Pubmed]
  18. PPARs and the complex journey to obesity. Evans, R.M., Barish, G.D., Wang, Y.X. Nat. Med. (2004) [Pubmed]
  19. Peroxisome biogenesis: advances and conundrums. Lazarow, P.B. Curr. Opin. Cell Biol. (2003) [Pubmed]
  20. mPPAR gamma 2: tissue-specific regulator of an adipocyte enhancer. Tontonoz, P., Hu, E., Graves, R.A., Budavari, A.I., Spiegelman, B.M. Genes Dev. (1994) [Pubmed]
  21. Peroxisome assembly factor-2, a putative ATPase cloned by functional complementation on a peroxisome-deficient mammalian cell mutant. Tsukamoto, T., Miura, S., Nakai, T., Yokota, S., Shimozawa, N., Suzuki, Y., Orii, T., Fujiki, Y., Sakai, F., Bogaki, A., Yasumo, H., Osumi, T. Nat. Genet. (1995) [Pubmed]
  22. Nuclear receptors in macrophage biology: at the crossroads of lipid metabolism and inflammation. Castrillo, A., Tontonoz, P. Annu. Rev. Cell Dev. Biol. (2004) [Pubmed]
  23. Peroxisomal membrane ghosts in Zellweger syndrome--aberrant organelle assembly. Santos, M.J., Imanaka, T., Shio, H., Small, G.M., Lazarow, P.B. Science (1988) [Pubmed]
  24. Presence of cytoplasmic factors functional in peroxisomal protein import implicates organelle-associated defects in several human peroxisomal disorders. Wendland, M., Subramani, S. J. Clin. Invest. (1993) [Pubmed]
  25. Induction and origin of hepatocytes in rat pancreas. Reddy, J.K., Rao, M.S., Qureshi, S.A., Reddy, M.K., Scarpelli, D.G., Lalwani, N.D. J. Cell Biol. (1984) [Pubmed]
  26. Drosophila ultraspiracle modulates ecdysone receptor function via heterodimer formation. Yao, T.P., Segraves, W.A., Oro, A.E., McKeown, M., Evans, R.M. Cell (1992) [Pubmed]
  27. Activation of human aortic smooth-muscle cells is inhibited by PPARalpha but not by PPARgamma activators. Staels, B., Koenig, W., Habib, A., Merval, R., Lebret, M., Torra, I.P., Delerive, P., Fadel, A., Chinetti, G., Fruchart, J.C., Najib, J., Maclouf, J., Tedgui, A. Nature (1998) [Pubmed]
  28. Activation of a member of the steroid hormone receptor superfamily by peroxisome proliferators. Issemann, I., Green, S. Nature (1990) [Pubmed]
  29. Human PEX7 encodes the peroxisomal PTS2 receptor and is responsible for rhizomelic chondrodysplasia punctata. Braverman, N., Steel, G., Obie, C., Moser, A., Moser, H., Gould, S.J., Valle, D. Nat. Genet. (1997) [Pubmed]
  30. PEX12 encodes an integral membrane protein of peroxisomes. Okumoto, K., Fujiki, Y. Nat. Genet. (1997) [Pubmed]
  31. The Pas2 protein essential for peroxisome biogenesis is related to ubiquitin-conjugating enzymes. Wiebel, F.F., Kunau, W.H. Nature (1992) [Pubmed]
  32. Bile acid profiles in peroxisomal 3-oxoacyl-coenzyme A thiolase deficiency. Clayton, P.T., Patel, E., Lawson, A.M., Carruthers, R.A., Collins, J. J. Clin. Invest. (1990) [Pubmed]
  33. A new self-assembled peroxisomal vesicle required for efficient resealing of the plasma membrane. Jedd, G., Chua, N.H. Nat. Cell Biol. (2000) [Pubmed]
  34. Evidence for a new human CYP1A1 regulation pathway involving PPAR-alpha and 2 PPRE sites. Sérée, E., Villard, P.H., Pascussi, J.M., Pineau, T., Maurel, P., Nguyen, Q.B., Fallone, F., Martin, P.M., Champion, S., Lacarelle, B., Savouret, J.F., Barra, Y. Gastroenterology (2004) [Pubmed]
  35. The PAL1 gene product is a peroxisomal ATP-binding cassette transporter in the yeast Saccharomyces cerevisiae. Swartzman, E.E., Viswanathan, M.N., Thorner, J. J. Cell Biol. (1996) [Pubmed]
  36. Subcellular localization of sterol carrier protein-2 in rat hepatocytes: its primary localization to peroxisomes. Keller, G.A., Scallen, T.J., Clarke, D., Maher, P.A., Krisans, S.K., Singer, S.J. J. Cell Biol. (1989) [Pubmed]
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