title: Use of mixed dispersion of fluorescent galactosylceramide and sodium dodecylsulfate for assaying galactosylceramide-beta-galactosidase and diagnosing Krabbe disease.
abstract: A fluorescent derivative of galactosylceramide [NBD-GalCer: 12-N-methyl-N-(7-nitrobenz-2-oxa-1,3-diazol-4-yl)aminodecanoyl+ ++ galactosylceramide] was dispersed in sodium dodecylsulfate and used to determine the activity of galactosylceramide-beta-galactosidase. Optimal assay conditions were defined, compared to other assay procedures and found suitable for diagnosing Krabbe disease.
mesh_terms: D009327:4-Chloro-7-nitrobenzofurazan / Q000031:analogs & derivatives; D002554:Cerebrosides; D005696:Galactosidases / Q000032:analysis*; D005698:Galactosylceramidase / Q000032:analysis* / Q000172:deficiency; D005699:Galactosylceramides; D006801:Humans; D007965:Leukodystrophy, Globoid Cell / Q000175:diagnosis* / Q000201:enzymology; D017830:Octoxynol; D010069:Oxadiazoles; D011092:Polyethylene Glycols; D012967:Sodium Dodecyl Sulfate; D013050:Spectrometry, Fluorescence
chemical_list: D002554:Cerebrosides; D005699:Galactosylceramides; C046928:NBD-galactosylceramide; D010069:Oxadiazoles; D012967:Sodium Dodecyl Sulfate; D011092:Polyethylene Glycols; D017830:Octoxynol; D005696:Galactosidases; D005698:Galactosylceramidase; D009327:4-Chloro-7-nitrobenzofurazan