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Title: Quantification of psychosine in the serum of twitcher mouse by LC-ESI-tandem-MS analysis
Authors: Zanfini, A.
Dreassi, Elena 
Berardi, A.
Governini, L.
Corbini, Gianfranco 
Costantino Ceccarini, E.
Balestri, Paolo
Luddi, Alice
Issue Date: 2013
Project: None 
Globoid cell leukodystrophy or Krabbe disease is an inherited autosomal recessive disorder caused bymutations in the galactosylceramidase (GALC) gene. Deficiency of GALC results in the accumulation ofa highly cytotoxic metabolite galactosylsphingosine (psychosine). In the present study, we describe thedevelopment and validation of a sensitive and specific LC–ESI-tandem-MS method for the determinationof psychosine in the serum of twitcher mice, the naturally occurring animal model of this disease. Themethod was validated in terms of accuracy, precision, specificity, linearity and sensitivity. Calibrationplots were linear over the concentration range of 2.5–50 ng/mL. Recovery of psychosine from serum wasin the range 94.20–98.02%. The results of this study show that in the affected mice the concentration ofpsychosine (ranging from 2.53 to 33.27 ng/mL) increased significantly with the progression of the disease.The maximum level (33.27 ng/mL) was detected in the serum of one of the twitcher mice sacrificedat 40 PND. Psychosine was not detected at significant levels in wild type mice. These results clearlydemonstrate that this noninvasive, rapid, and highly sensitive LC–ESI-tandem-MS method is a very usefulapproach for the detection of psychosine in serum.
ISSN: 0731-7085
DOI: 10.1016/j.jpba.2013.02.039
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