The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
MeSH Review

Zellweger Syndrome

Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of Zellweger Syndrome


High impact information on Zellweger Syndrome


Chemical compound and disease context of Zellweger Syndrome


Biological context of Zellweger Syndrome


Anatomical context of Zellweger Syndrome


Gene context of Zellweger Syndrome


  1. PER3, a gene required for peroxisome biogenesis in Pichia pastoris, encodes a peroxisomal membrane protein involved in protein import. Liu, H., Tan, X., Russell, K.A., Veenhuis, M., Cregg, J.M. J. Biol. Chem. (1995) [Pubmed]
  2. Plasmalogen biosynthesis in peroxisomal disorders: fatty alcohol versus alkylglycerol precursors. Schrakamp, G., Schalkwijk, C.G., Schutgens, R.B., Wanders, R.J., Tager, J.M., van den Bosch, H. J. Lipid Res. (1988) [Pubmed]
  3. Cerebro-hepato-renal (Zellweger) syndrome, adrenoleukodystrophy, and Refsum's disease: plasma changes and skin fibroblast phytanic acid oxidase. Poulos, A., Sharp, P., Fellenberg, A.J., Danks, D.M. Hum. Genet. (1985) [Pubmed]
  4. Very long chain fatty acids in genetic peroxisomal disease fibroblasts: differences between the cerebro-hepato-renal (Zellweger) syndrome and adrenoleukodystrophy variants. Molzer, B., Korschinsky, M., Bernheimer, H., Schmid, R., Wolf, C., Roscher, A. Clin. Chim. Acta (1986) [Pubmed]
  5. Polarizing inclusions in some organs of children with congenital peroxisomal diseases (Zellweger's, Refsum's, chondrodysplasia punctata (rhizomelic form), X-linked adrenoleukodystrophy). Kerckaert, I., Dingemans, K.P., Heymans, H.S., Vamecq, J., Roels, F. J. Inherit. Metab. Dis. (1988) [Pubmed]
  6. Hydroxyeicosatetraenoic acid metabolism in cultured human skin fibroblasts. Evidence for peroxisomal beta-oxidation. Gordon, J.A., Figard, P.H., Spector, A.A. J. Clin. Invest. (1990) [Pubmed]
  7. 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]
  8. Disturbed cholesterol homeostasis in a peroxisome-deficient PEX2 knockout mouse model. Kovacs, W.J., Shackelford, J.E., Tape, K.N., Richards, M.J., Faust, P.L., Fliesler, S.J., Krisans, S.K. Mol. Cell. Biol. (2004) [Pubmed]
  9. Neuronal migration disorder in Zellweger mice is secondary to glutamate receptor dysfunction. Gressens, P., Baes, M., Leroux, P., Lombet, A., Van Veldhoven, P., Janssen, A., Vamecq, J., Marret, S., Evrard, P. Ann. Neurol. (2000) [Pubmed]
  10. Globoid cells, glial nodules, and peculiar fibrillary changes in the cerebro-hepato-renal syndrome of Zellweger. de León, G.A., Grover, W.D., Huff, D.S., Morinigo-Mestre, G., Punnett, H.H., Kistenmacher, M.L. Ann. Neurol. (1977) [Pubmed]
  11. Pipecolic acid is oxidized by renal and hepatic peroxisomes. Implications for Zellweger's cerebro-hepato-renal syndrome (CHRS). Zaar, K., Angermüller, S., Völkl, A., Fahimi, H.D. Exp. Cell Res. (1986) [Pubmed]
  12. Severe deficiency of docosahexaenoic acid in peroxisomal disorders: a defect of delta 4 desaturation? Martínez, M. Neurology (1990) [Pubmed]
  13. Pathologic alterations in the brain and liver in hyperpipecolic acidemia. Challa, V.R., Geisinger, K.R., Burton, B.K. J. Neuropathol. Exp. Neurol. (1983) [Pubmed]
  14. Plasma and skin fibroblast C26 fatty acids in infantile Refsum's disease. Poulos, A., Sharp, P. Neurology (1984) [Pubmed]
  15. The occurrence of polyenoic very long chain fatty acids with greater than 32 carbon atoms in molecular species of phosphatidylcholine in normal and peroxisome-deficient (Zellweger's syndrome) brain. Poulos, A., Sharp, P., Johnson, D., Easton, C. Biochem. J. (1988) [Pubmed]
  16. Plasma polyenoic very-long-chain fatty acids in peroxisomal disease: biochemical discrimination of Zellweger's syndrome from other phenotypes. Poulos, A., Sharp, P., Johnson, D. Neurology (1989) [Pubmed]
  17. Structural and chemical alterations in the cerebral maldevelopment of fetal cerebro-hepato-renal (Zellweger) syndrome. Powers, J.M., Tummons, R.C., Caviness, V.S., Moser, A.B., Moser, H.W. J. Neuropathol. Exp. Neurol. (1989) [Pubmed]
  18. Complementation study of peroxisome-deficient disorders by immunofluorescence staining and characterization of fused cells. Yajima, S., Suzuki, Y., Shimozawa, N., Yamaguchi, S., Orii, T., Fujiki, Y., Osumi, T., Hashimoto, T., Moser, H.W. Hum. Genet. (1992) [Pubmed]
  19. Cerebro-hepato-renal (Zellweger) syndrome and neonatal adrenoleukodystrophy: similarities in phenotype and accumulation of very long chain fatty acids. Brown, F.R., McAdams, A.J., Cummins, J.W., Konkol, R., Singh, I., Moser, A.B., Moser, H.W. The Johns Hopkins medical journal. (1982) [Pubmed]
  20. Sterol carrier protein-2 is involved in cholesterol transfer from the endoplasmic reticulum to the plasma membrane in human fibroblasts. Puglielli, L., Rigotti, A., Greco, A.V., Santos, M.J., Nervi, F. J. Biol. Chem. (1995) [Pubmed]
  21. Age-related differences in plasmalogen content of erythrocytes from patients with the cerebro-hepato-renal (Zellweger) syndrome: implications for postnatal detection of the disease. Wanders, R.J., Purvis, Y.R., Heymans, H.S., Bakkeren, J.A., Parmentier, G.G., van Eldere, J., Eyssen, H., van den Bosch, H., Tager, J.M., Schutgens, R.B. J. Inherit. Metab. Dis. (1986) [Pubmed]
  22. A prenatal test for the cerebro-hepato-renal (Zellweger) syndrome by demonstration of the absence of catalase-containing particles (peroxisomes) in cultured amniotic fluid cells. Wanders, R.J., Schrakamp, G., van den Bosch, H., Tager, J.M., Schutgens, R.B. Eur. J. Pediatr. (1986) [Pubmed]
  23. Stereological analysis of peroxisomes and mitochondria in intestinal epithelium of patients with peroxisomal deficiency disorders: Zellweger's syndrome and neonatal-onset adrenoleukodystrophy. Black, V.H., Cornacchia, L. Am. J. Anat. (1986) [Pubmed]
  24. Adrenoleukodystrophy: survey of 303 cases: biochemistry, diagnosis, and therapy. Moser, H.W., Moser, A.E., Singh, I., O'Neill, B.P. Ann. Neurol. (1984) [Pubmed]
  25. Neuronal migration depends on intact peroxisomal function in brain and in extraneuronal tissues. Janssen, A., Gressens, P., Grabenbauer, M., Baumgart, E., Schad, A., Vanhorebeek, I., Brouwers, A., Declercq, P.E., Fahimi, D., Evrard, P., Schoonjans, L., Collen, D., Carmeliet, P., Mannaerts, G., Van Veldhoven, P., Baes, M. J. Neurosci. (2003) [Pubmed]
  26. Docosahexaenoic acid deficit is not a major pathogenic factor in peroxisome-deficient mice. Janssen, A., Baes, M., Gressens, P., Mannaerts, G.P., Declercq, P., Van Veldhoven, P.P. Lab. Invest. (2000) [Pubmed]
  27. Caudothalamic groove cysts in Zellweger syndrome. Unay, B., Kendirli, T., Ataç, K., Gül, D., Akin, R., Gökçay, E. Clin. Dysmorphol. (2005) [Pubmed]
  28. Zellweger syndrome and associated phenotypes. FitzPatrick, D.R. J. Med. Genet. (1996) [Pubmed]
WikiGenes - Universities