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Peroxisome Mosaics in the Liver of Patients and the Regulation of Peroxisome Expression in Rat Hepatocyte Cultures a
Author(s) -
ROELS FRANK,
TYTGAT TOM,
BEKEN SONJA,
GIROS MARISA,
ESPEEL MARC,
PREST BETTY,
KERCKAERT INGRID,
PÀMPOLS T.,
ROGIERS VERA
Publication year - 1996
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1111/j.1749-6632.1996.tb18640.x
Subject(s) - embryology , library science , humanities , pathology , medicine , anatomy , philosophy , computer science
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