期刊
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
卷 1588, 期 3, 页码 195-202出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/S0925-4439(02)00165-5
关键词
ceruloplasmin; iron overload; divalent metal transporter 1; ferroportin 1; hephaestin; transferrin receptor
Aceruloplasminemia is an autosomal recessive disorder caused by mutations in the ceruloplasmin (CP) gene, and is characterized by a unique combination of neurovisceral iron overload and iron deficiency anemia. We generated CP-deficient (CP-/-) mice to investigate the functional involvement of CP in iron metabolism. The mice showed a marked iron overload in the liver and mild iron deficiency anemia. We examined the expression of iron-metabolism genes in the duodenum and liver using TaqMan RT-PCR. The divalent metal transporter 1 (DMTI), ferroportin 1 (FPN1), and hephaestin (HEPH) genes were not up-regulated in the duodenum from CP-/- mice. These data suggest that the mechanism of hepatic iron overload in aceruloplasminemia is quite different from that in hemochromatoses and atransferrinemia. In the liver, CP-/- mice showed no increase of gene expression for DMTI and transferrin receptors (TFR and TFR2), indicating that none of the known pathways of iron uptake is activated in hepatocytes of CP-/- mice. This result supports the hypothesis that CP mainly acts to release iron from cells in the liver. (C) 2002 Elsevier Science B.V. All rights reserved.
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