Psychosine, the cytotoxic sphingolipid that accumulates in globoid cell leukodystrophy, alters membrane architecture
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چکیده
منابع مشابه
Aberrant production of tenascin-C in globoid cell leukodystrophy alters psychosine-induced microglial functions.
Globoid cell leukodystrophy (GLD), or Krabbe disease, is a rare and often fatal demyelinating disease caused by mutations in the galactocerebrosidase (galc) gene that result in accumulation of galactosylsphingosine (psychosine). We recently reported that the extracellular matrix (ECM) protease, matrix metalloproteinase-3, is elevated in GLD and that it regulates psychosine-induced microglial ac...
متن کامل[Globoid cell leukodystrophy].
A 29 year old male with onset of globoid cell leukodystrophy at age 14 is described. This is the first case of enzymatically confirmed globoid cell leukodystrophy with onset of symptoms after the age of ten. This patient is unique because of the late onset and slow progression and extends the clinical spectrum of globoid cell leukodystrophy. Globoid cell leukodystrophy is caused by the deficien...
متن کاملMMP-3 mediates psychosine-induced globoid cell formation: implications for leukodystrophy pathology.
Globoid cell leukodystrophy (GLD) or Krabbe disease, is a fatal demyelinating disease attributed to mutations in the galactocerebrosidase (GALC) gene. Loss of function mutations in GALC result in accumulation of the glycolipid intermediate, galactosylsphingosine (psychosine). Due to the cytotoxicity of psychosine, it has been hypothesized that accumulated psychosine underlie the pathophysiology...
متن کاملGloboid cell leukodystrophy (GLD) is a rapidly progress- ing pediatric neurodegenerative disease caused by missing or dysfunctional lysosomal enzyme galactosylceramidase
Journal of Lipid Research Volume 54, 2013 3303 Copyright © 2013 by the American Society for Biochemistry and Molecular Biology, Inc. Globoid cell leukodystrophy (GLD) is a rapidly progressing pediatric neurodegenerative disease caused by missing or dysfunctional lysosomal enzyme galactosylceramidase (GALC). Hallmarks of this disease include macrophage (globoid cell) infi ltration into the brain...
متن کاملPsychosine accumulates in membrane microdomains in the brain of krabbe patients, disrupting the raft architecture.
Lipid rafts (LRs) are membrane realms characterized by high concentrations of cholesterol and sphingolipids. Often, they are portrayed as scaffolds on which many different signaling molecules can assemble their cascades. The idea of rafts as scaffolds is garnering significant attention as the consequences of LR disruption have been shown to be manifest in multiple signaling pathways. In this st...
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ژورنال
عنوان ژورنال: Molecular Genetics and Metabolism
سال: 2013
ISSN: 1096-7192
DOI: 10.1016/j.ymgme.2012.11.108