Article
Comparative study on glucocerebrosidase in spleens from patients with Gaucher disease.
The Biochemical journal - 1 Jul 1990
Aerts J M, Donker-Koopman W E, Brul S, Van Weely S, Sa Miranda M C, Barranger J A, Tager J M, Schram A W
Abstract excerpt
In Gaucher disease (glucosylceramide lipidosis), deficiency of glucocerebrosidase causes pathological storage of glucosylceramide, particularly in the spleen. A comparative biochemical and immunological analysis has therefore been made of glucocerebrosidase in spleens from normal subjects (n = 4) and from Gaucher disease patients with non-neuronopathic (n = 5) and neuronopathic (n = 5) phenotypes. The spleens...
Topics
- Blood Platelets
- Gaucher Disease
- Glucosidases
- Glucosylceramidase
- Glycoproteins
- Glycosylation
- Humans
- Hydrolases
- Immunoblotting
- Immunosorbent Techniques
- Isoelectric Point