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Correction of the copper transport defect of Menkes patient fibroblasts by expression of two forms of the sheep Wilson ATPase

Lockhart, Paul J., La Fontaine, Sharon, Firth, Stephen D., Greenough, Mark, Camakaris, James and Mercer, Julian 2002, Correction of the copper transport defect of Menkes patient fibroblasts by expression of two forms of the sheep Wilson ATPase, Biochimica et biophysica acta (BBA) - molecular basis of disease, vol. 1588, no. 2, pp. 189-194, doi: 10.1016/S0925-4439(02)00164-3.

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Title Correction of the copper transport defect of Menkes patient fibroblasts by expression of two forms of the sheep Wilson ATPase
Author(s) Lockhart, Paul J.
La Fontaine, SharonORCID iD for La Fontaine, Sharon orcid.org/0000-0002-9948-074X
Firth, Stephen D.
Greenough, Mark
Camakaris, James
Mercer, Julian
Journal name Biochimica et biophysica acta (BBA) - molecular basis of disease
Volume number 1588
Issue number 2
Start page 189
End page 194
Publisher Elsevier Science BV
Place of publication Netherlands
Publication date 2002-11-20
ISSN 0925-4439
Keyword(s) copper metabolism
P-type ATPase
ATP7B
Summary The Wilson disease (WD) protein (ATP7B) is a copper-transporting P-type ATPase that is responsible for the efflux of hepatic copper into the bile, a process that is essential for copper homeostasis in mammals. Compared with other mammals, sheep have a variant copper phenotype and do not efficiently excrete copper via the bile, often resulting in excessive copper accumulation in the liver. To investigate the function of sheep ATP7B and its potential role in the copper-accumulation phenotype, cDNAs encoding the two forms of ovine ATP7B were transfected into immortalised fibroblast cell lines derived from a Menkes disease patient and a normal control. Both forms of ATP7B were able to correct the copper-retention phenotype of the Menkes cell line, demonstrating each to be functional copper-transporting molecules and suggesting that the accumulation of copper in the sheep liver is not due to a defect in the copper transport function of either form of sATP7B.
Language eng
DOI 10.1016/S0925-4439(02)00164-3
Field of Research 060499 Genetics not elsewhere classified
HERDC Research category C1 Refereed article in a scholarly journal
Copyright notice ©2002 Elsevier Science B.V.
Persistent URL http://hdl.handle.net/10536/DRO/DU:30001737

Document type: Journal Article
Collection: School of Biological and Chemical Sciences
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