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  • 2005-2009  (1)
  • 1980-1984  (4)
  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    Journal of the European Academy of Dermatology and Venereology 19 (2005), S. 0 
    ISSN: 1468-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: We report the case of a 42-year-old man with pseudoxanthoma elasticum (PXE) and recurrent bilateral nephrolithiasis. Diagnosis of PXE was made by yellow papules on the neck and ophthalmologic angioid streaks. This diagnosis was confirmed by a skin biopsy (Von Kossa stain) and by genotyping analysis of ABCC6 (homozygous mutation R1138Q). Nephrolithiasis was recurrent and biological investigations showed hypophosphoraemia, hyperphosphaturia, hypercalciuria, normocalcaemia, normal serum parathyroid hormone value, high 1,25-dihydroxy vitamin D value and a renal calcium oxalate stone. ABCC6 encodes for MRP6, a multidrug resistant protein strongly expressed in the liver and kidney. The substrates of the MRP6 remain unknown. As PXE is characterized by calcification of elastic fibres and this patient presents important phosphocalcic anomalies, we discuss the possible implications of MRP6 in the phosphocalcic metabolism.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-119X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary The localization of β2-microglobulin (β2m) was studied in renal biopsies from 18 patients with pathological transplanted kidney using immunofluorescence and electron-immunohistochemical techniques; the renal biopsies of 4 cases with normal kidneys were used as controls. Using immunofluorescence, β2m was not observed in the normal kidneys, β2m was found in the glomeruli (7 cases) and the tubular epithelium (16 cases) of the transplanted kidneys. Using immunoelectron microscopy, some labelling of the normal kidneys was observed mainly along the cell coat of foot processes and in tubular-epithelial lysosomes. In the glomeruli of transplanted kidneys, particularly in cases of acute or chronic rejection, β2m was most frequently localized on the outer layer of the basement membrane and along the cell coat of foot processes. The brush border of the proximal tubules and the lysosomal structures were intensely labelled. Although immunoelectron-microscopy studies are unable to discriminate between the localization of β2m in normal and transplanted kidneys, these findings nevertheless suggest the glomerular filtration of β2m and its metabolism in the tubular epithelium.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1434-6036
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Abstract TheE g andT 2g acoustic phonon modes of the molecular crystals KBr0.96(CN)0.04 and KBr0.86(CN)0.14 have been investigated by inelastic neutron scattering at 6, 90 and 300 K. Clear resonances due to mode-mode coupling to molecular excitations were observed. In complementary ultrasonic experiments the elastic constants were found to pass through minima as a function of temperature. The results were quantitavely explained by a model which assumes simple forms of the molecular excitation spectrum. An alternative interpretation, though being only qualitative, postulates a freezing of the CN molecules into a glass-like phase.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0584
    Keywords: Amyloidosis ; Monoclonal protein ; Immunoglobulins
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Among 55 amyloidoses, the detection of a monoclonal protein (MP) led to the selection of 15 primary and 3 myeloma-associated types of amyloidosis. Therefore the presence of a MP gives evidence for an immunocytic amyloidosis. The λ-light-chain nature of MP and the abundant production of free light-chains are two of the factors predisposing to the production of amyloid deposits (AL) in the course of immunocyte dyscrasias.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    New York : Wiley-Blackwell
    Journal of Polymer Science: Polymer Chemistry Edition 21 (1983), S. 2945-2946 
    ISSN: 0360-6376
    Keywords: Physics ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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