Reviewer's report
Title:A Wilson protein expression, copper excretion and sweat production in
sweat glands of Wilson disease patients and controls
Version: 1Date:18 March 2008
Reviewer: Marc Solioz
Reviewer's report:
The paper by Schaefer et al. describes analysis of the expression of the Wilson
copper ATPase in skin of normal and LEC rats as well as in human skin biopsies.
It was found that the Wilson protein is expressed in human and rat sweat gland
epithelia. Copper was measured in sweat and found to be not significantly
different in controls and Wilson patients. It was also described that Wilson
disease patients had lower sweat production, which was partially reversed by
treatment or transplantation.
The expression of Wilson protein in sweat glands in controls and a lack of
expression in the LEC rats is convincingly demonstrated. There was no
correlation between Wilson protein expression and copper in sweat. Given the
methodology at hand, it is unfortunate that the authors did not look at copper in
sweat of brindled mice to determine if the Menkes copper-ATPase is responsible
for sweat copper. The conclusion that treatment of Wilson disease or
transplantation reverses the reduced sweat volume accompanying the disease is
based a single observation each, which is hardly convincing.
Major points:
Various and differing data points have been excluded from the plots of Figs. 2 to
4. There is no rationale for this.
Minor comments:
Throughout: 30 min, not 30min
Various places: penicillamine, trientine, zinc, etc. are not capitalized in English.
pg. 3, line 30: Correct grammar and spelling...by pilocarpin induced
ionotopheresis...
pg. 7, line 9: Wilson patient...was, not patients...were - this was a single case.
pg. 8, line 11: ...Menkes protein as copper-ATPase...correct grammar.
Fig. 1. The micrographs are missing scales in the pictures (fold magnification
does not account for the size of reproduction).
Fig. 3 and 4: Express sweat volumes in ul/min, not ul/30 min.
Fig. 5A and B. The axis are labeled with "Wilson patients", but in fact it was one
patient. Figures 5A and B are not justified. These five values can be described in
the text.
What next?: Accept after minor essential revisions
Level of interest: An article whose findings are important to those with closely
related research interests
Quality of written English: Acceptable
Statistical review: No, the manuscript does not need to be seen by a
statistician.
Declaration of competing interests:
I declare that I have no competing interests.
Reviewer's report
Title:A Wilson protein expression, copper excretion and sweat production in
sweat glands of Wilson disease patients and controls
Version: 1Date:18 March 2008
Reviewer: Marc Solioz
Reviewer's report:
The paper by Schaefer et al. describes analysis of the expression of the Wilson
copper ATPase in skin of normal and LEC rats as well as in human skin biopsies.
It was found that the Wilson protein is expressed in human and rat sweat gland
epithelia. Copper was measured in sweat and found to be not significantly
different in controls and Wilson patients. It was also described that Wilson
disease patients had lower sweat production, which was partially reversed by
treatment or transplantation.
The expression of Wilson protein in sweat glands in controls and a lack of
expression in the LEC rats is convincingly demonstrated. There was no
correlation between Wilson protein expression and copper in sweat. Given the
methodology at hand, it is unfortunate that the authors did not look at copper in
sweat of brindled mice to determine if the Menkes copper-ATPase is responsible
for sweat copper. The conclusion that treatment of Wilson disease or
transplantation reverses the reduced sweat volume accompanying the disease is
based a single observation each, which is hardly convincing.
Major points:
Various and differing data points have been excluded from the plots of Figs. 2 to
4. There is no rationale for this.
Minor comments:
Throughout: 30 min, not 30min
Various places: penicillamine, trientine, zinc, etc. are not capitalized in English.
pg. 3, line 30: Correct grammar and spelling...by pilocarpin induced
ionotopheresis...
pg. 7, line 9: Wilson patient...was, not patients...were - this was a single case.
pg. 8, line 11: ...Menkes protein as copper-ATPase...correct grammar.
Fig. 1. The micrographs are missing scales in the pictures (fold magnification
does not account for the size of reproduction).
Fig. 3 and 4: Express sweat volumes in ul/min, not ul/30 min.
Fig. 5A and B. The axis are labeled with "Wilson patients", but in fact it was one
patient. Figures 5A and B are not justified. These five values can be described in
the text.
What next?: Accept after minor essential revisions
Level of interest: An article whose findings are important to those with closely
related research interests
Quality of written English: Acceptable
Statistical review: No, the manuscript does not need to be seen by a
statistician.
Declaration of competing interests:
I declare that I have no competing interests.
Please review this paper.
user
Reviewer's report
Title:A Wilson protein expression, copper excretion and sweat production in
sweat glands of Wilson disease patients and controls
Version: 1Date:18 March 2008
Reviewer: Marc Solioz
Reviewer's report:
The paper by Schaefer et al. describes analysis of the expression of the Wilson
copper ATPase in skin of normal and LEC rats as well as in human skin biopsies.
It was found that the Wilson protein is expressed in human and rat sweat gland
epithelia. Copper was measured in sweat and found to be not significantly
different in controls and Wilson patients. It was also described that Wilson
disease patients had lower sweat production, which was partially reversed by
treatment or transplantation.
The expression of Wilson protein in sweat glands in controls and a lack of
expression in the LEC rats is convincingly demonstrated. There was no
correlation between Wilson protein expression and copper in sweat. Given the
methodology at hand, it is unfortunate that the authors did not look at copper in
sweat of brindled mice to determine if the Menkes copper-ATPase is responsible
for sweat copper. The conclusion that treatment of Wilson disease or
transplantation reverses the reduced sweat volume accompanying the disease is
based a single observation each, which is hardly convincing.
Major points:
Various and differing data points have been excluded from the plots of Figs. 2 to
4. There is no rationale for this.
Minor comments:
Throughout: 30 min, not 30min
Various places: penicillamine, trientine, zinc, etc. are not capitalized in English.
pg. 3, line 30: Correct grammar and spelling...by pilocarpin induced
ionotopheresis...
pg. 7, line 9: Wilson patient...was, not patients...were - this was a single case.
pg. 8, line 11: ...Menkes protein as copper-ATPase...correct grammar.
Fig. 1. The micrographs are missing scales in the pictures (fold magnification
does not account for the size of reproduction).
Fig. 3 and 4: Express sweat volumes in ul/min, not ul/30 min.
Fig. 5A and B. The axis are labeled with "Wilson patients", but in fact it was one
patient. Figures 5A and B are not justified. These five values can be described in
the text.
What next?: Accept after minor essential revisions
Level of interest: An article whose findings are important to those with closely
related research interests
Quality of written English: Acceptable
Statistical review: No, the manuscript does not need to be seen by a
statistician.
Declaration of competing interests:
I declare that I have no competing interests.
assistant