title: Globotriaosylceramide isoform profiles in human plasma by liquid chromatography-tandem mass spectrometry.
abstract: 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.
authors.affiliation: Genzyme Corporation, Immunology, One Mountain Road, Framingham, MA 01701-93222, USA.
authors.forename: Bryant C
authors.initials: BC
authors.lastname: Nelson
authors.forename: Thomas
authors.initials: T
authors.lastname: Roddy
authors.forename: Shaparak
authors.initials: S
authors.lastname: Araghi
authors.forename: Dennis
authors.initials: D
authors.lastname: Wilkens
authors.forename: John J
authors.initials: JJ
authors.lastname: Thomas
authors.forename: Kate
authors.initials: K
authors.lastname: Zhang
authors.forename: Crystal C-C
authors.initials: CC
authors.lastname: Sung
authors.forename: Susan M
authors.initials: SM
authors.lastname: Richards
mesh_terms: D002853:Chromatography, Liquid / Q000379:methods*; D006801:Humans; D007536:Isomerism; D021241:Spectrometry, Mass, Electrospray Ionization / Q000379:methods*; D014281:Trihexosylceramides / Q000097:blood*
chemical_list: D014281:Trihexosylceramides; C018549:globotriaosylceramide