Human genetic defect in leukotriene C4 synthesis

Normal human platelets metabolise [3H]-LTA4 into [3H]-LTC4. Platelets from patients with glutathione synthetase deficiency possessing 10-30% of normal levels of cellular glutathione showed marked reduction in capacity to form [3H]-LTC4 (8-10% of normal) even though exogenous reduced glutathione was...

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Veröffentlicht in:	Biochemical and biophysical research communications 1986-11, Vol.140 (3), p.857-860
Hauptverfasser:	PACE-ASCIAK, C. R, KLEIN, J, SPIELBERG, S. P
Format:	Artikel
Sprache:	eng
Schlagworte:	Applied sciences Arachidonic Acids - blood Blood Platelets - enzymology Child Exact sciences and technology Glutathione - metabolism Glutathione Synthase - deficiency Glutathione Synthase - genetics Humans In Vitro Techniques Leukotriene A4 Male Metabolism, Inborn Errors - genetics Other techniques and industries SRS-A - biosynthesis SRS-A - genetics
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Beschreibung
Zusammenfassung:	Normal human platelets metabolise [3H]-LTA4 into [3H]-LTC4. Platelets from patients with glutathione synthetase deficiency possessing 10-30% of normal levels of cellular glutathione showed marked reduction in capacity to form [3H]-LTC4 (8-10% of normal) even though exogenous reduced glutathione was added to the incubation medium. To our knowledge this is the first demonstration of a genetic defect in LTC4 synthetase coupled to a defect in cellular glutathione levels.
ISSN:	0006-291X 1090-2104
DOI:	10.1016/0006-291X(86)90713-8