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  • 1
    Electronic Resource
    Electronic Resource
    Pathology of hepatic peroxisomes and mitochondria in patients with peroxisomal disorders (1990)
    Springer
    Virchows Archiv 416 (1990), S. 255-264 
    Details
    ISSN: 1432-2307
    Keywords: Peroxisomes ; Peroxisomal disorders ; Neonatal adrenoleukodystrophy ; Infantile Refsum's disease ; Pseudo-Zellweger disease ; Mitochondria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The morphology of hepatic peroxisomes in five patients with metabolic disorders believed to be due to inherited defects of peroxisomal function or biogenesis is described. Electron microscopy and cytochemical staining for catalase were used to identify peroxisomes in two boys with infantile Refsum's disease (IRD), a girl with autopsy confirmed neonatal adrenoleukodystrophy (NALD), and two boys with pseudo-Zellweger syndrome (PZS). In the patients with IRD and NALD hepatic peroxisomes were significantly reduced in size and number and contained electron dense centres. In the liver of the patients with PZS the peroxisomes were enlarged. Morphologically abnormal peroxisomes were also detected in autopsy tissue from one boy with PZS using electron microscopy. Lamellar-lipid inclusions and mitochondria with crystalline inclusions and/or abnormal cristae are also described in two patients, one with IRD, the other with NALD.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01678985
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Ultrastructure and immunocytochemistry of hepatic peroxisomes in rhizomelic chondrodysplasia punctata (1992)
    Springer
    European journal of pediatrics 151 (1992), S. 829-836 
    Details
    ISSN: 1432-1076
    Keywords: Peroxisomes ; Peroxisomal disorders ; Rhizomelic chondrodysplasia punctata ; Zellweger syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Peroxisomes were studied in the liver of two rhizomelic chondrodysplasia punctata patients using electron microscopy and catalase cytochemistry. Immuno-electron microscopy was carried out on the liver of one of these patients using antibodies to catalase, acyl-CoA oxidase, bifunctional protein, 3-ketoacyl-CoA thiolase and a 68 kDa peroxisomal membrane protein, in conjunction with protein-A colloidal gold. Moderately to markedly enlarged, flocculent peroxisomes were found in both patients. In one patient they were very heterogeneous with regard to the number per hepatocyte. The peroxisomes had very low levels of catalase as indicated by cytochemistry and immunocytochemistry. The three β-oxidation enzymes were localised normally within the peroxisomes. The 68 kDa membrane protein was localised to the peroxisomal membranes. Some extra membrane loops were also identified using this antibody.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957935
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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