HOMEPRODUCTSSERVICESCOMPANYCONTACTFAQResearchDictionaryPharmaMobileSign Up FREE or Login

Sulfogalactocerebroside and bis-(monoacylglyceryl)-phosphate as activators of spleen glucocerebrosidase.

Abstract
Sequential extraction of human spleen membranes with sodium cholate and n-butanol removes endogenous lipids and renders glucocerebrosidase activity dependent upon exogenous acidic lipids (e.g., phosphatidylserine, gangliosides) and a heat-stable activator protein (HSF). In the present report, we show that two previously untested lysosomal acidic lipids, namely sulfogalactocerebroside and bis-(monoacylglyceryl)-phosphate (BMP), also activate normal human glucocerebrosidase. In addition, sulfogalactocerebroside also markedly enhanced the activity of glucocerebrosidase isolated from a patient with type 1 (non-neuronopathic) Gaucher's disease, resulting in a specific activity which was 60-80% that of control glucocerebrosidase. Furthermore, when the sulfolipid was used as the activator, glucocerebrosidase from the type 1 patient was 30 times more active than the corresponding glucocerebrosidase from a person with type 2 (neuronopathic) Gaucher's disease. In contrast, the two BMPs, one rich in C26 saturated fatty acid and another rich in C18 unsaturated fatty acids, were relatively poor activators of both mutant glucocerebrosidases while providing excellent reconstitution of control activity.
AuthorsE M Prence, K O Garrett, H Panitch, A Basu, R H Glew, J R Wherrett, S Huterer
JournalClinica chimica acta; international journal of clinical chemistry (Clin Chim Acta) Vol. 156 Issue 2 Pg. 179-89 (Apr 30 1986) ISSN: 0009-8981 [Print] NETHERLANDS
PMID3085988 (Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Cerebrosides
  • Galactosylceramides
  • Lysophospholipids
  • Monoglycerides
  • Phosphatidic Acids
  • Phosphatidylserines
  • bis(monoacylglyceryl)phosphate
  • sulfogalactocerebroside
  • Glucosidases
  • beta-Glucosidase
  • Glucosylceramidase
Topics
  • Animals
  • Cerebrosides (pharmacology)
  • Child
  • Enzyme Activation (drug effects)
  • Galactosylceramides (pharmacology)
  • Gaucher Disease (enzymology)
  • Glucosidases (metabolism)
  • Glucosylceramidase (metabolism)
  • Humans
  • Lysophospholipids
  • Lysosomes (analysis)
  • Monoglycerides
  • Phosphatidic Acids (pharmacology)
  • Phosphatidylserines (pharmacology)
  • Rats
  • Spleen (enzymology)
  • beta-Glucosidase (metabolism)

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research network!


Choose Username:
Email:
Password:
Verify Password: