BACKGROUND We retrospectively compared biochemical responses in type 1 Gaucher disease patients to treatment with glycosphingolipid synthesis inhibitors miglustat and eliglustat and ERT. METHODS Seventeen GD1 patients were included (n = 6 eliglustat, (two switched from ERT), n = 9 miglustat (seven switchers), n = 4 ERT (median dose 60U/kg/m). Plasma protein markers reflecting disease burden (...

The neutral glycosphingolipids and the gangliosides of normal 3T3 mouse fibroblasts, one polyoma-transformed, and three SV40-transformed clonal isolates have been examined. The normal 3T3 cells contained monoand dihexosylceramides and a complex pattern of gangliosides. The carbohydrate and fatty acid compositions of these neutral glycosphingolipids and of the principal gangliosides were determi...

Luminescence thermal sensing and deep-tissue imaging using nanomaterials operating within the first biological window (ca. 700-980 nm) are of great interest, prompted by the ever-growing demands in the fields of nanotechnology and nanomedicine. Here, we show that (Gd1-xNdx)2O3 (x = 0.009, 0.024 and 0.049) nanorods exhibit one of the highest thermal sensitivity and temperature uncertainty report...

This study examined the effect of prenatal perfluorooctanoic acid (PFOA) administration on pre- and postnatal development using peroxisome proliferator-activated receptor α (PPARα)-humanized mice to determine if species differences in receptor activity might influence the developmental effects induced by PFOA. Pregnant mice were treated daily with water or PFOA (3mg/kg) by po gavage from gestat...

Gangliosides, a member of the glycosphingolipid family, are heterogeneously expressed in biological membranes and are particularly enriched within the central nervous system. Gangliosides consist of mono- or poly-sialylated oligosaccharide chains of variable lengths attached to a ceramide unit and are found to be intimately involved in brain disease development. The purpose of this study is to ...

Salivary gland homogenate (SGH) from the female mosquitoes Anopheles gambiae, An. stephensi, An. freeborni, An. dirus and An. albimanus were found to exhibit hemagglutinating (lectin) activity. Lectin activity was not found for male An. gambiae, or female Ae aegypti, Culex quinquefasciatus, Phlebotomus duboscqi, and Lutzomyia longipalpis. With respect to species-specificity, An. gambiae SGH agg...