Peroxisome Mosaics in the Liver of Patients and the Regulation of Peroxisome Expression in Rat Hepatocyte Cultures<sup>a</sup>

Roels, Frank; Tytgat, Tom; Beken, Sonja; Girós, M.; Espeel, Marc; Prest, B. De; Kerckaert, Ingrid; Pàmpols, T; Rogiers, Vera

doi:10.1111/j.1749-6632.1996.tb18640.x

Cited by 16 publications

(8 citation statements)

References 18 publications

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“…In other patients, a mosaic distribution of parenchymal cells with and without peroxisomes was found (Espeel et al, 1995b;Giros et al, 1995;Mandel et al, 1994;Roels et al, 1996a). Several hypotheses have been proposed to explain the mosaic distribution, for example, that the microenvironment (extracellular matrix) influences the differentiation of the peroxisome population.…”

Section: Hepatic Peroxisomes In Peroxisomal Disorder Patients In Relamentioning

confidence: 90%

“…This hypothesis was inferred from the facts that (1) hepatocyte-specific features are determined by tissue interactions early during development (e.g. Caron, 1990;Cascio and Zaret, 1991) and (2) the number, size, shape, and catalase activity of peroxisomes in cultured hepatocytes depend on the culture conditions (Furukawa et al, 1988;Mitaka et al, 1993;Roels et al, 1996a). Thus far, studies of early embryonic rat liver have not confirmed a relationship between peroxisome maturation and the presence of extracellular matrix proteins laminin and collagen IV (Depreter et al, 1997).…”

Section: Hepatic Peroxisomes In Peroxisomal Disorder Patients In Relamentioning

confidence: 97%

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Biogenesis of peroxisomes in fetal liver

Espeel

Depreter

Nardacci

et al. 1997

Microsc. Res. Tech.

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Section: Hepatic Peroxisomes In Peroxisomal Disorder Patients In Relamentioning

confidence: 90%

Section: Hepatic Peroxisomes In Peroxisomal Disorder Patients In Relamentioning

confidence: 97%

Biogenesis of peroxisomes in fetal liver

Espeel

Depreter

Nardacci

et al. 1997

Microsc. Res. Tech.

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“…The parenchyma without Px displayed the same features as the first group of liver samples: there was cytoplasmic and nuclear catalase and AGT in the hepatocytes, and macrophage lysosomes contained trilamellar inclusions and acetone-insoluble lipid droplets (Espeel et al, 1995a(Espeel et al, , 1998bMandel et al, 1994;Pineda et al, 1999;Roels et al, 1995aRoels et al, , 1996a.…”

Section: Peroxisomal Mosaics (10 Patients)mentioning

confidence: 87%

“…In control livers the minimal axial ratio is 0.41. Elongated Px are constitutive in human duodenal epithelium (Roels et al, 1991a); they also make their appearance when hepatocytes are cultured, which is considered to reflect dedifferentiation (Depreter et al, 2000a;Roels et al, 1996a;Schrader et al, 1996). 7.…”

Section: Microscopic Pathology and Cytochemistrymentioning

confidence: 99%

Human peroxisomal disorders

Depreter

Espeel

Roels

2003

Microscopy Res & Technique

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Peroxisomes are single membrane-bound cell organelles performing numerous metabolic functions. The present article aims to give an overview of our current knowledge about inherited peroxisomal disorders in which these organelles are lacking or one or more of their functions are impaired. They are multiorgan disorders and the nervous system is implicated in most. After a summary of the historical names and categories, each having distinct symptoms and prognosis, microscopic pathology is reviewed in detail. Data from the literature are added to experience in the authors' laboratory with 167 liver biopsy and autopsy samples from peroxisomal patients, and with a smaller number of chorion samples for prenatal diagnosis, adrenal-, kidney-, and brain samples. Various light and electron microscopic methods are used including enzyme- and immunocytochemistry, polarizing microscopy, and morphometry. Together with other laboratory investigations and clinical data, this approach continues to contribute to the diagnosis and further characterization of peroxisomal disorders, and the discovery of novel variants. When liver specimens are examined, three main groups including 9 novel variants (33 patients) are distinguished: (1) absence or (2) presence of peroxisomes, and (3) mosaic distribution of cells with and without peroxisomes (10 patients). Renal microcysts, polarizing trilamellar inclusions, and insoluble lipid in macrophages in liver, adrenal cortex, brain, and in interstitial cells of kidney are also valuable for classification. On a genetic basis, complementation of fibroblasts has classified peroxisome biogenesis disorders into 12 complementation groups. Peroxisome biogenesis genes (PEX), knock-out-mice, and induction of redundant genes are briefly reviewed, including some recent results with 4-phenylbutyrate. Finally, regulation of peroxisome expression during development and in cell cultures, and by physiological factors is discussed.

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“…Mosaicism is another possible explanation for variations in phenotypic expression. Five PBD patients with relatively mild phenotypes have been reported in which the import defect showed variable expression in different tissues, or within the same tissue (33)(34)(35)(36). At the cellular level these patients show variability of expression between the liver and cultured skin fibroblasts.…”

Section: Genotype-phenotype Correlations In the Disorders Of Peroxisomentioning

confidence: 99%

Molecular genetics of peroxisomal disorders

Moser¹

2000

Front Biosci

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Peroxisome Mosaics in the Liver of Patients and the Regulation of Peroxisome Expression in Rat Hepatocyte Cultures^a

Cited by 16 publications

References 18 publications

Biogenesis of peroxisomes in fetal liver

Biogenesis of peroxisomes in fetal liver

Human peroxisomal disorders

Molecular genetics of peroxisomal disorders

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Peroxisome Mosaics in the Liver of Patients and the Regulation of Peroxisome Expression in Rat Hepatocyte Culturesa

Cited by 16 publications

References 18 publications

Biogenesis of peroxisomes in fetal liver

Biogenesis of peroxisomes in fetal liver

Human peroxisomal disorders

Molecular genetics of peroxisomal disorders

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Product

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Peroxisome Mosaics in the Liver of Patients and the Regulation of Peroxisome Expression in Rat Hepatocyte Cultures^a