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Peroxisome mosaics in the liver of patients and the regulation of peroxisome expression in rat hepatocyte cultures

Author
Organization
Abstract
Peroxisomal deficiency disorders, which are genetically transmitted, are assumed to be expressed in all cells, and the use of cultured skin fibroblasts for diagnosis and research is based on this assumption. We describe four unrelated patients, three boys and a girl, with clinical, biochemical and microscopic evidence of a peroxisomal disorder whose livers display mosaicism, that is, parenchymal cells with peroxisomes are adjacent to cells without peroxisomes. After discussing the possible origin of these mosaics, we examine the influence of the environment on the expression of peroxisomes in adult rat hepatocytes in primary monolayer and three-dimensional culture. In this model the expression of peroxisomes varies between cells and depends upon the culture conditions.
Keywords
CATALASE, ACID, INFANTILE REFSUM-DISEASE, PATHOLOGY, CONFIGURATION

Citation

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Chicago
Roels, Frank, Tom Tytgat, S Beken, Marisa Giros, Marc Espeel, Betty De Prest, Ingrid Kerckaert, T Pampols, and V Rogiers. 1996. “Peroxisome Mosaics in the Liver of Patients and the Regulation of Peroxisome Expression in Rat Hepatocyte Cultures.” Ed. JK Reddy, T Suga, GP Mannaerts, PB Lazarow, and S Subrammani. Annals of the New York Academy of Sciences 804: 502–515.
APA
Roels, F., Tytgat, T., Beken, S., Giros, M., Espeel, M., De Prest, B., Kerckaert, I., et al. (1996). Peroxisome mosaics in the liver of patients and the regulation of peroxisome expression in rat hepatocyte cultures. (J. Reddy, T. Suga, G. Mannaerts, P. Lazarow, & S. Subrammani, Eds.)Annals of the New York Academy of Sciences, 804, 502–515. Presented at the International symposium on Peroxisomes : Biology and role in toxicology and disease.
Vancouver
1.
Roels F, Tytgat T, Beken S, Giros M, Espeel M, De Prest B, et al. Peroxisome mosaics in the liver of patients and the regulation of peroxisome expression in rat hepatocyte cultures. Reddy J, Suga T, Mannaerts G, Lazarow P, Subrammani S, editors. Annals of the New York Academy of Sciences. New York, NY, USA: New York Academy of Sciences; 1996;804:502–15.
MLA
Roels, Frank, Tom Tytgat, S Beken, et al. “Peroxisome Mosaics in the Liver of Patients and the Regulation of Peroxisome Expression in Rat Hepatocyte Cultures.” Ed. JK Reddy et al. Annals of the New York Academy of Sciences 804 (1996): 502–515. Print.
@article{182334,
  abstract     = {Peroxisomal deficiency disorders, which are genetically transmitted, are assumed to be expressed in all cells, and the use of cultured skin fibroblasts for diagnosis and research is based on this assumption. We describe four unrelated patients, three boys and a girl, with clinical, biochemical and microscopic evidence of a peroxisomal disorder whose livers display mosaicism, that is, parenchymal cells with peroxisomes are adjacent to cells without peroxisomes. After discussing the possible origin of these mosaics, we examine the influence of the environment on the expression of peroxisomes in adult rat hepatocytes in primary monolayer and three-dimensional culture. In this model the expression of peroxisomes varies between cells and depends upon the culture conditions.},
  author       = {Roels, Frank and Tytgat, Tom and Beken, S and Giros, Marisa and Espeel, Marc and De Prest, Betty and Kerckaert, Ingrid and Pampols, T and Rogiers, V},
  editor       = {Reddy, JK and Suga, T and Mannaerts, GP and Lazarow, PB and Subrammani, S},
  issn         = {0077-8923},
  journal      = {Annals of the New York Academy of Sciences},
  keyword      = {CATALASE,ACID,INFANTILE REFSUM-DISEASE,PATHOLOGY,CONFIGURATION},
  language     = {eng},
  location     = {Aspen, CO, USA},
  pages        = {502--515},
  publisher    = {New York Academy of Sciences},
  title        = {Peroxisome mosaics in the liver of patients and the regulation of peroxisome expression in rat hepatocyte cultures},
  url          = {http://dx.doi.org/10.1111/j.1749-6632.1996.tb18640.x},
  volume       = {804},
  year         = {1996},
}

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