Decrease of PKC precedes other cellular signs of calpain activation after transient cerebral ischemia.
In PLoS ONE, 2002
... (Covance, Emeryville, CA, 1∶500), mouse monoclonal anti-MAP2 (Sigma, St Louis, MO, 1∶500), mouse monoclonal anti-galactocerebroside (GalC) (Chemicon, Temecula, CA, 1∶200) and anti S100beta (Swant, Bellinzona, Switzerland, 1∶1000) ...
Experimental therapies in the murine model of globoid cell leukodystrophy.
Saint Louis, United States. In Pediatr Neurol, 2014
BACKGROUND: Globoid cell leukodystrophy or Krabbe disease, is a rapidly progressive childhood lysosomal storage disorder caused by a deficiency in galactocerebrosidase. Galactocerebrosidase deficiency leads to the accumulation of galactosylsphingosine (psychosine), a cytotoxic lipid especially damaging to oligodendrocytes and Schwann cells.
Diagnostic difficulties in Krabbe disease: a report of two cases and review of literature.
Warsaw, Poland. In Folia Neuropathol, 2011
Globoid cell leukodystrophy (GLD, also known as Krabbe disease), whose pathophysiology is still not completely elucidated, is an inherited, metabolic, and neurodegenerative disease, caused by the deficiency of β-galactocerebrosidase (GALC) or in very rare cases by lack of active saposin A. We describe two patients, in whom first MRI changes were not suggestive of GLD.
Progressive accumulation of toxic metabolite in a genetic leukodystrophy.
In Science, 1984
Progressive accumulation of a cytotoxic metabolite, galactosylsphingosine (psychosine), was found in the brain of the twitcher mouse, a mutant caused by genetic deficiency of galactosylceramidase. Similar abnormal accumulation was also found in the brain of the genetic galactosylceramidase deficiency disease in the dog and in human patients (globoid cell leukodystrophy or Krabbe disease).
Enzyme replacement in grafted nerve of twitcher mouse.
In Nature, 1983
In the experiments reported here, we have attempted to determine whether the morphological improvement of grafted twitcher nerves is accompanied by an increase in activity of the enzyme galactosylceramidase.