asialo-gm1-ganglioside and Gaucher-Disease

asialo-gm1-ganglioside has been researched along with Gaucher-Disease* in 1 studies

Other Studies

1 other study(ies) available for asialo-gm1-ganglioside and Gaucher-Disease

ArticleYear
Glycolipid analysis of different tissues and cerebrospinal fluid in type II Gaucher disease.
    Journal of inherited metabolic disease, 2002, Volume: 25, Issue:1

    The lipid composition or the liver, spleen, brain, cerebellum and cerebrospinal fluid of a Gaucher disease type II patient who died at the age of 5 months was examined. The glycolipid analysis demonstrated a marked increase of total amounts not only in the peripheral tissues but also in the brain cerebellum and cerebrospinal fluid, with a prevalence of glucosylceramide. A reduction in gangliosides was observed in all the analysed tissues with a relative increase of GD3 in the nervous tissue. The fatty acid composition of glucosylceramide showed a prevalence of stearic acid in the central nervous system, while in the peripheral tissues palmitic acid was prevalent. This result suggests a different origin of the glucosylceramide stored in different tissues. The generalized reduction of gangliosides and their modified distribution together with the central nervous system GD3 increment represent a new observation. These data could be useful in the effort to clarify the pathophysiological mechanism of brain damage in neuronopathic Gaucher disease.

    Topics: Brain; Brain Chemistry; Cerebellum; Female; G(M1) Ganglioside; Gangliosides; Gaucher Disease; Glucosylceramides; Glycolipids; Humans; Infant; Lactosylceramides; Liver; Spleen

2002