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  • 1
    Electronic Resource
    Electronic Resource
    Protease Inhibitors Suppress the Degradation of Mutant Adrenoleukodystrophy Proteins but Do Not Correct Impairment of Very Long Chain Fatty Acid Metabolism in Adrenoleukodystrophy Fibroblasts (1997)
    Springer
    Neurochemical research 22 (1997), S. 233-237 
    Details
    ISSN: 1573-6903
    Keywords: Adrenoleukodystrophy ; turnover ; protease inhibitor ; very long chain fatty acid ; β-oxidation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The adrenoleukodystrophy (ALD) gene product, ALD protein (ALDP), was not detected in fibro-blasts from our or most other patients with ALD as determined by immunoblot or immunocyto-chemistry. We investigated the stability of mutant ALDP and found from pulse-chase experiments that the respective half-lives of the normal and mutant #140 (Gly512Ser) and #249 (Arg660Trp) were 72.6, 32.1 and 26.1 min, indicative that mutant ALDPs are less stable than normal ones. The mutant ALDPs were detectable in fibroblasts cultured with the protease inhibitor E-64 or leupeptin. Protease inhibitor treatment for 2 to 28 days did not affect the amount of very long chain fatty acid (VLCFA), C26:0, or VLCFA β-oxidation activity in ALD fibroblasts. Protease inhibitors therefore suppress the degradation of ALDP but do not correct the impairment of VLCFA metabolism in ALD.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1023/A:1022477001703
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Ceramide Induces Apoptosis to Immature Cerebellar Granule Cells in Culture (1999)
    Springer
    Neurochemical research 24 (1999), S. 685-690 
    Details
    ISSN: 1573-6903
    Keywords: Programmed cell death ; sphingomyelin ; neurons ; neuronal death
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A recent study revealed that ceramide acts as a second messenger in the sphingomyelin pathway and thus plays an important regulatory role in programmed cell death (apoptosis) to cell the lines induced by tumor-necrosis factor (TNF)-α and interleukin (IL)-1β, although its effect remains controversial regarding primary neuronal culture. We investigated the effect of a cell-permeable ceramide analog (C2-ceramide) on cultures of cerebellar granule cells, which is thought to have active sphingomyelin pathway during development. The presence of C2-ceramide decreased the number of cerebellar granule cells (CGCs) in a concentration-dependent manner when added at DIV 1 (1 day in vitro). The ED50 was 60 μM. After DIV2, CGCs became less sensitive to C2-ceramide and the ED50 was 200 μM at DIV 7. DNA staining with Hoechst 33258 showed the morphology of apoptotic nuclei in the degenerating neurons. Internucleosomal DNA degradation could also be observed by gel electrophoresis. Protein and RNA synthesis inhibitors prevented the death of neurons. C2-dihydroceramide, which lacks the 4–5 trans double bond and failed to induce neuronal death. These results thus demonstrated that C2-ceramide induces apoptosis to the CGCs at the early stage in vitro, however the CGCs were found to be less sensitive to C2-ceramide at the later stage in vitro.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1023/A:1021008626887
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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