Globotriaosylceramide isoform profiles in human plasma by liquid chromatography-tandem mass spectrometry.
Globotriaosylceramide (GL3) is a heterogeneous glycosphingolipid that is elevated in the blood plasma of patients diagnosed with Fabry disease. GL3 consists of numerous isoforms, some of which are distinctly specific to human plasma. An electrospray-ionization LC/MS/MS method has been developed that has the capacity to monitor the GL3 isoform profiles in plasma extracts. Total GL3 is extracted from human plasma via chloroform/methanol liquid-liquid extraction, purified by C(18) solid-phase extraction and analyzed by multiple reaction monitoring LC/MS/MS. The relative responses of eight selected isoforms are calculated on the basis of the total GL3 response and the isoform responses are subsequently utilized to construct isoform profile plots.
Nelson
Bryant C
BC
Genzyme Corporation, Immunology, One Mountain Road, Framingham, MA 01701-93222, USA.
Roddy
Thomas
T
Araghi
Shaparak
S
Wilkens
Dennis
D
Thomas
John J
JJ
Zhang
Kate
K
Sung
Crystal C-C
CC
Richards
Susan M
SM
D002853:Chromatography, Liquid / Q000379:methods*; D006801:Humans; D007536:Isomerism; D021241:Spectrometry, Mass, Electrospray Ionization / Q000379:methods*; D014281:Trihexosylceramides / Q000097:blood*
D014281:Trihexosylceramides; C018549:globotriaosylceramide