A method for measuring globotriaosylceramide (Gb3, or GL3) levels in plasma and urine of humans affected by Anderson-Fabry disease has been developed. The analyses are performed using flow injection analysis-electrospray ionization-tandem mass spectrometry (FIA-ESI-MS/MS). The method is rapid, sensitive and hence suitable for high-throughput analyses, requiring only a simple 50-fold dilution for the preparation of plasma and urine samples. The detection of the analytes of interest was achieved using a triple quadrupole instrument operating in the multiple reaction monitoring mode. The linearity of the calibration standard responses, the intra- and inter-assay precision, the accuracy and the detection limit of the method were evaluated. The proposed method allows a rapid and accurate assessment of globotriaosylceramide in biological samples. Data obtained from healthy volunteers and Anderson-Fabry affected subjects suggest a potential role for this technique in monitoring the effectiveness of Anderson-Fabry disease therapy. The results obtained in two actual cases treated with enzyme replacement therapy are reported and discussed.

Rapid quantitation of globotriaosylceramide in human plasma and urine: a potential application for monitoring enzyme replacement therapy in Anderson-Fabry disease / F. Boscaro; G. Pieraccini; G. la Marca; G. Bartolucci; C. Luceri; F. Luceri; G. Moneti. - In: RAPID COMMUNICATIONS IN MASS SPECTROMETRY. - ISSN 0951-4198. - STAMPA. - 16:(2002), pp. 1507-1514.

Rapid quantitation of globotriaosylceramide in human plasma and urine: a potential application for monitoring enzyme replacement therapy in Anderson-Fabry disease

PIERACCINI, GIUSEPPE;LA MARCA, GIANCARLO;BARTOLUCCI, GIAN LUCA;LUCERI, CRISTINA;MONETI, GLORIANO
2002

Abstract

A method for measuring globotriaosylceramide (Gb3, or GL3) levels in plasma and urine of humans affected by Anderson-Fabry disease has been developed. The analyses are performed using flow injection analysis-electrospray ionization-tandem mass spectrometry (FIA-ESI-MS/MS). The method is rapid, sensitive and hence suitable for high-throughput analyses, requiring only a simple 50-fold dilution for the preparation of plasma and urine samples. The detection of the analytes of interest was achieved using a triple quadrupole instrument operating in the multiple reaction monitoring mode. The linearity of the calibration standard responses, the intra- and inter-assay precision, the accuracy and the detection limit of the method were evaluated. The proposed method allows a rapid and accurate assessment of globotriaosylceramide in biological samples. Data obtained from healthy volunteers and Anderson-Fabry affected subjects suggest a potential role for this technique in monitoring the effectiveness of Anderson-Fabry disease therapy. The results obtained in two actual cases treated with enzyme replacement therapy are reported and discussed.
2002
16
1507
1514
F. Boscaro; G. Pieraccini; G. la Marca; G. Bartolucci; C. Luceri; F. Luceri; G. Moneti
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/253277
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