Wilson protein expression, copper excretion and sweat production in sweat glands of Wilson disease patients and controls.
BACKGROUND
In Wilson disease, copper is not sufficiently excreted into bile due to the absence or malfunction of the Wilson protein copper ATPase in the excretory pathway of hepatocytes. Copper is found in sweat. It is unknown if the Wilson protein plays a role in copper excretion into sweat. It is the aim of this study to investigate Wilson protein expression in sweat glands and analysing its effects on copper excretion into sweat in controls and patients with Wilson disease.


METHODS
Immunofluorescent analysis of the Wilson protein in skin samples from normal rat, LEC rat and human skin biopsies were performed. Pilocarpin-induced sweat gland stimulation by iontophoretic transfer adapted from the methods used for cystic fibrosis sweat test was used for sweat induction. Sweat volume, sweat copper concentration, serum ceruloplasmin and serum copper were analysed in 28 Wilson patients and 21 controls.


RESULTS
The Wilson protein is expressed in human and rat sweat gland epithelia. Copper concentration in sweat is not significantly different between controls and Wilson patients. Wilson patients produce significantly smaller volumes of sweat compared to controls. Sweat production is partially reversible in Wilson patients under medical treatment for Wilson disease or after liver transplantation


CONCLUSIONS
Wilson patients show a reduced sweat production with unaltered sweat copper concentration. The Wilson protein might play an important role in physiological sweat production.
Schaefer
Mark
M
Department of Gastroenterology and Infections Diseases, University of Heidelberg Medical School, INF 410, D-69120 Heidelberg, Germany. kontakt@dr-schaefer-praxis.de
Schellenberg
Mavi
M
Merle
Uta
U
Weiss
Karl Heinz
KH
Stremmel
Wolfgang
W
D000251:Adenosine Triphosphatases / Q000378:metabolism*; D000328:Adult; D000818:Animals; D027682:Cation Transport Proteins / Q000378:metabolism*; D003300:Copper / Q000378:metabolism*; D000073840:Copper-Transporting ATPases; D005260:Female; D005455:Fluorescent Antibody Technique; D006527:Hepatolenticular Degeneration / Q000378:metabolism / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D008297:Male; D009994:Osmolar Concentration; D051381:Rats; D011922:Rats, Mutant Strains; D020127:Recovery of Function; D013542:Sweat / Q000378:metabolism*; D013545:Sweat Glands / Q000378:metabolism*; D013546:Sweating
D027682:Cation Transport Proteins; D003300:Copper; D000251:Adenosine Triphosphatases; D000073840:Copper-Transporting ATPases