Mode of action of triethylenetetramine dihydrochloride on copper metabolism in Wilson's disease.
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.
Siegemund
R
R
Paul Flechsig Institute for Brain Research, Department of Neuro-chemistry, Clinic of Leipzig University, Germany.
Lössner
J
J
Günther
K
K
Kühn
H J
HJ
Bachmann
H
H
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
D013431:Sulfates; D019287:Zinc Sulfate; D003300:Copper; D010396:Penicillamine; D015032:Zinc; D014266:Trientine