|1.||Grabowski, Gregory A: 8 articles (12/2015 - 03/2010)|
|2.||Sun, Ying: 8 articles (12/2015 - 03/2010)|
|3.||Liou, Benjamin: 6 articles (12/2015 - 03/2010)|
|4.||Xu, You-Hai: 5 articles (12/2015 - 03/2010)|
|5.||Setchell, Kenneth D R: 5 articles (12/2015 - 01/2011)|
|6.||Zhang, Wujuan: 5 articles (12/2015 - 01/2011)|
|7.||Sidransky, Ellen: 4 articles (09/2011 - 08/2002)|
|8.||Dasgupta, Nupur: 3 articles (12/2015 - 01/2013)|
|9.||Boot, Rolf G: 3 articles (04/2015 - 10/2011)|
|10.||Ferraz, Maria J: 3 articles (04/2015 - 10/2011)|
|1.||Gaucher Disease (Gaucher's Disease)
10/23/2015 - "Glucosylsphingosine (GluSph) has emerged as a biomarker for the inherited metabolic disorder, Gaucher disease (GD). "
04/01/2015 - "Abnormalities in urinary GlcSph are a hallmark of Gaucher disease allowing biochemical confirmation of diagnosis. "
09/01/2013 - "Mice with Gaucher's disease showed visceral storage of β-glucosylceramide and greatly elevated plasma β-glucosylsphingosine [median 57.9 (range 19.8-159) nm; n = 39] compared with control mice from the same strain [median 0.56 (range 0.04-1.38) nm; n = 29] (p < 0.0001). "
01/01/2013 - "Glucosylsphingosine is a highly sensitive and specific biomarker for primary diagnostic and follow-up monitoring in Gaucher disease in a non-Jewish, Caucasian cohort of Gaucher disease patients."
01/01/2013 - "These results demonstrate age, organ, and mutation-specific quantitative differences in GC species and glucosylsphingosine accumulations that can have influence in the tissue/regional expression of Gaucher disease phenotypes."
|2.||Globoid Cell Leukodystrophy (Krabbe Disease)
07/01/2003 - "The application and optimization of this technique for lyso-n-GSLs and lyso-GSLs will permit their quantification in small amounts of biological tissues, particularly in the GSL storage diseases, such as Gaucher and Krabbe's disease, in which GlcSph and GalSph, respectively, accumulate."
09/01/2011 - "To facilitate the study of the chemical pathology of galactosylsphingosine (psychosine, GalSph) in Krabbe disease and glucosylsphingosine (GlcSph) in Gaucher disease, we have devised a facile method for the effective separation of these two glycosylsphingosines from other glycosphingolipids (GSLs) in Krabbe brain and Gaucher spleen samples. "
|3.||Lysosomal Storage Diseases (Lysosomal Storage Disease)
08/01/2014 - "Gaucher disease, a prevalent lysosomal storage disease (LSD), is caused by insufficient activity of acid β-glucosidase (GCase) and the resultant glucosylceramide (GC)/glucosylsphingosine (GS) accumulation in visceral organs (Type 1) and the central nervous system (Types 2 and 3). "
01/01/2014 - "Gaucher disease is a lysosomal storage disease caused by defective activity of acid β-glucosidase (GCase), which leads to the accumulation of its major substrates, glucosylceramide (GlcCer) and glucosylsphingosine (GlcSph) in many cells. "
|4.||Fabry Disease (Fabry's Disease)
05/01/2014 - "The value of elevated glucosylsphingosine in Gaucher disease and globotriaosylsphingosine in Fabry disease for diagnosis and monitoring of disease is discussed as well as the possible contribution of the sphingoid bases to (patho)physiology. "
04/01/2015 - "In analogy to globotriaosylsphingsone in urine of Fabry disease patients, additional isoforms of GlcSph differing in structure of the sphingosine moiety were identified in GD urine samples. "
|9.||Protein Isoforms (Isoforms)
|10.||Reactive Oxygen Species (Oxygen Radicals)