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

Elizabeth M. Prence, Kevin O. Garrett, Howard Panitch, Alakananda Basu, Robert H. Glew, John R. Wherrett, Srebrenka Huterer

Research output: Contribution to journalArticle

5 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)179-189
Number of pages11
JournalClinica Chimica Acta
Volume156
Issue number2
DOIs
StatePublished - 30 Apr 1986

Fingerprint

Glucosylceramidase
Spleen
Gaucher Disease
Lipids
Sodium Cholate
1-Butanol
Gangliosides
Phosphatidylserines
Unsaturated Fatty Acids
bis(monoacylglyceryl)phosphate
sulfogalactocerebroside
Fatty Acids
Hot Temperature
Membranes

Keywords

  • Gaucher's disease
  • Glucocerebrosidase
  • Heat-stable factor
  • Sphingolipidosis
  • Sulfogalactocerebroside
  • bis-(Monoacylglyceryl)-phosphate

Cite this

Prence, Elizabeth M. ; Garrett, Kevin O. ; Panitch, Howard ; Basu, Alakananda ; Glew, Robert H. ; Wherrett, John R. ; Huterer, Srebrenka. / Sulfogalactocerebroside and bis-(monoacylglyceryl)-phosphate as activators of spleen glucocerebrosidase. In: Clinica Chimica Acta. 1986 ; Vol. 156, No. 2. pp. 179-189.
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Sulfogalactocerebroside and bis-(monoacylglyceryl)-phosphate as activators of spleen glucocerebrosidase. / Prence, Elizabeth M.; Garrett, Kevin O.; Panitch, Howard; Basu, Alakananda; Glew, Robert H.; Wherrett, John R.; Huterer, Srebrenka.

In: Clinica Chimica Acta, Vol. 156, No. 2, 30.04.1986, p. 179-189.

Research output: Contribution to journalArticle

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T1 - Sulfogalactocerebroside and bis-(monoacylglyceryl)-phosphate as activators of spleen glucocerebrosidase

AU - Prence, Elizabeth M.

AU - Garrett, Kevin O.

AU - Panitch, Howard

AU - Basu, Alakananda

AU - Glew, Robert H.

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