title: Mode of action of triethylenetetramine dihydrochloride on copper metabolism in Wilson's disease.
abstract: The drug of choice for the initial treatment of "decoppering" in Wilson's disease, an inherited disorder of copper metabolism, is the chelating agent D-penicillamine. In the case of harmful side-effects an alternative drug is triethylenetetramine dihydrocholoride (trien or trientine). Using the 24-h-urine excretion of copper and the oral copper loading test with copper-64, a double function for trien was found: trien increases the urine copper excretion and decreases the intestinal copper absorption respectively.
authors.affiliation: Paul Flechsig Institute for Brain Research, Department of Neuro-chemistry, Clinic of Leipzig University, Germany.
authors.forename: R
authors.initials: R
authors.lastname: Siegemund
authors.forename: J
authors.initials: J
authors.lastname: Lössner
authors.forename: K
authors.initials: K
authors.lastname: Günther
authors.forename: H J
authors.initials: HJ
authors.lastname: Kühn
authors.forename: H
authors.initials: H
authors.lastname: Bachmann
mesh_terms: D000328:Adult; D003300:Copper / Q000652:urine*; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D006527:Hepatolenticular Degeneration / Q000188:drug therapy* / Q000652:urine; D006801:Humans; D008297:Male; D008875:Middle Aged; D010396:Penicillamine / Q000627:therapeutic use; D013431:Sulfates / Q000627:therapeutic use; D014266:Trientine / Q000627:therapeutic use*; D015032:Zinc / Q000627:therapeutic use; D019287:Zinc Sulfate
chemical_list: D013431:Sulfates; D019287:Zinc Sulfate; D003300:Copper; D010396:Penicillamine; D015032:Zinc; D014266:Trientine