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Haemolysis and perturbations in the systemic iron metabolism of suckling, copper-deficient mosaic mutant mice : an animal model of menkes disease

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Haemolysis and perturbations in the systemic iron metabolism of suckling, copper-deficient mosaic mutant mice : an animal model of menkes disease

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dc.contributor.author Lenartowicz, Małgorzata [SAP11012906] pl
dc.contributor.author Starzyński, Rafał R. pl
dc.contributor.author Krzeptowski, Wojciech [SAP12019715] pl
dc.contributor.author Grzmil, Paweł [SAP11016319] pl
dc.contributor.author Bednarz, Aleksandra [SAP14046483] pl
dc.contributor.author Ogórek, Mateusz [USOS113346] pl
dc.contributor.author Haberkiewicz, Olga [USOS96042] pl
dc.contributor.author Staroń, Robert pl
dc.contributor.author Gajowiak, Anna pl
dc.contributor.author Lipiński, Paweł pl
dc.date.accessioned 2015-04-29T13:06:33Z
dc.date.available 2015-04-29T13:06:33Z
dc.date.issued 2014 pl
dc.identifier.uri http://ruj.uj.edu.pl/xmlui/handle/item/6165
dc.language eng pl
dc.rights Udzielam licencji. Uznanie autorstwa 4.0 Międzynarodowa *
dc.rights.uri http://creativecommons.org/licenses/by/4.0/pl/legalcode *
dc.subject.other superoxide dysmutase pl
dc.subject.other ferroportin pl
dc.subject.other oxidative stress pl
dc.subject.other Ceruloplasmin pl
dc.title Haemolysis and perturbations in the systemic iron metabolism of suckling, copper-deficient mosaic mutant mice : an animal model of menkes disease pl
dc.type JournalArticle pl
dc.description.additional Olga Haberkiewicz podpisana: Olga Pierzchała pl
dc.abstract.other The biological interaction between copper and iron is best exemplified by the decreased activity of multicopper ferroxidases under conditions of copper deficiency that limits the availability of iron for erythropoiesis. However, little is known about how copper deficiency affects iron homeostasis through alteration of the activity of other copper-containing proteins, not directly connected with iron metabolism, such as superoxide dismutase 1 (SOD1). This antioxidant enzyme scavenges the superoxide anion, a reactive oxygen species contributing to the toxicity of iron via the Fenton reaction. Here, we analyzed changes in the systemic iron metabolism using an animal model of Menkes disease: copper-deficient mosaic mutant mice with dysfunction of the ATP7A copper transporter. We found that the erythrocytes of these mutants are copper-deficient, display decreased SOD1 activity/expression and have cell membrane abnormalities. In consequence, the mosaic mice show evidence of haemolysis accompanied by haptoglobin-dependent elimination of haemoglobin (Hb) from the circulation, as well as the induction of haem oxygenase 1 (HO1) in the liver and kidney. Moreover, the hepcidin-ferroportin regulatory axis is strongly affected in mosaic mice. These findings indicate that haemolysis is an additional pathogenic factor in a mouse model of Menkes diseases and provides evidence of a new indirect connection between copper deficiency and iron metabolism. pl
dc.description.volume 9 pl
dc.description.number 9 pl
dc.identifier.doi 10.1371/journal.pone.0107641 pl
dc.identifier.eissn 1932-6203 pl
dc.title.journal PLoS ONE pl
dc.language.container eng pl
dc.affiliation Wydział Biologii i Nauk o Ziemi : Instytut Zoologii pl
dc.subtype Article pl
dc.identifier.articleid e107641 pl
dc.rights.original CC-BY; otwarte czasopismo; ostateczna wersja wydawcy; w momencie opublikowania; 0; pl
dc.identifier.project ROD UJ / P pl
.pointsMNiSW [2014 A]: 40


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Udzielam licencji. Uznanie autorstwa 4.0 Międzynarodowa Except where otherwise noted, this item's license is described as Udzielam licencji. Uznanie autorstwa 4.0 Międzynarodowa