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lactosylceramidase
ceramide-Glc-Gal & H2O gives Cer-Glc & galactose; deficiency gives lactosylceramidosis; see also lactosylceramidase II which acts GM(1) ganglioside
Also Known As:
lactosylceramidase I; lactosylceramide galactosyl hydrolase
Networked:
4
relevant articles (
0
outcomes,
0
trials/studies)
Bio-Agent Context: Research Results
Enzymes and Coenzymes: 1
Enzymes: 152586
Hydrolases: 2166
Glycoside Hydrolases: 871
Galactosidases: 1473
Galactosylceramidase: 370
lactosylceramidase: 4
Related Diseases
1.
Globoid Cell Leukodystrophy (Krabbe Disease)
03/01/1977 - "
Lactosylceramidase assays for diagnosis of globoid cell leukodystrophy and GM1-gangliosidosis.
"
03/25/1975 - "
Normal human brain contains mostly lactosylceramidase I, while normal liver contains predominantly lactosylceramidase II. Lactosylceramidase I is genetically lacking globoid cell leukodystrophy, and lactosylceramidase II in GM1-gangliosidosis.
"
12/17/1975 - "
Tissues from patients with Krabbe's disease were found to be deficient in lactosylceramide beta-galactosidase activity (lactosylceramidase I) when pure taurocholate was used in the assay.
"
08/15/1987 - "
Lactosylsphingosine beta-galactosidase activities assayed in the absence and the presence of taurocholate (probably lactosylceramidase I) were deficient in fibroblasts from patients with globoid cell leukodystrophy, while the activity assayed with sodium cholate (probably lactosylceramidase II) was deficient in GM1 gangliosidosis fibroblasts.
"
2.
GM1 Gangliosidosis (Gangliosidosis GM1)
08/15/1987 - "
Lactosylsphingosine beta-galactosidase activities assayed in the absence and the presence of taurocholate (probably lactosylceramidase I) were deficient in fibroblasts from patients with globoid cell leukodystrophy, while the activity assayed with sodium cholate (probably lactosylceramidase II) was deficient in GM1 gangliosidosis fibroblasts.
"
Related Drugs and Biologics
1.
Taurocholic Acid (Sodium Taurocholate)
2.
acid beta-galactosidase
3.
Sodium Cholate
4.
beta-Galactosidase (Lactaid)
5.
lactosylceramide beta-galactosidase
6.
lactosylsphingosine