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Smith-Lemli-Opitz syndrome: phenotypic extreme with minimal clinical findings
Authors:MJ Nowaczyk  DT Whelan  RE Hill
Affiliation:Laboratory of Physiology and Pharmacology, School of Health Sciences, Faculty of Medicine, University of the Ryukyus, Okinawa, Japan.
Abstract:The effect of spine venom from the crown-of-thorns starfish (Acanthaster planci) on drug-metabolizing enzymes in rat liver was studied. The spine venom was prepared by saturation of spine homogenate with ammonium sulfate and the protein fraction precipitating 50% saturation was used as venom B. Venom A was the protein precipitated between 50 and 100% saturation. When venom B (100-200 mg/kg) was given to rats, liver microsomal GSH S-transferase and cytochrome P450 activities decreased while cytosolic GSH S-transferase activity was not changed. The decrease in these microsomal enzyme activities was seen from 12 hr to 24 hr after giving 100 mg/kg of venom B. Rats given venom A died, suggesting an involvement of the lethal factor in venom A. The data showed that the spine venom B from A. planci depressed microsomal GSH S-transferase and cytochrome P450 activities in rat liver and that this venom was distinct from the lethal factor of the spine venom.
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