Molecular analysis of iron overload in beta 2-microglobulin-deficient mice

• Type: Article in International Scientific Journal
• Date: 2004

Authors

Muckenthaler, MU (Author) Other; Rodrigues, P (Author) ICBAS; Macedo, MG (Author) Other; Minana, B (Author) Other; Brennan, K (Author) Other; Cardoso, EM (Author) Other; Hentze, MW (Author) Other; de Sousa, M (Author) Other

Journal

Title: Blood Cells, Molecules, and Diseases

Vol. 33

Pages: 125-131

ISSN: 1079-9796

Publisher: Elsevier

Indexing

ISI Web of Knowledge - 34 Citations

Scopus - 35 Citations

Scientific classification

FOS: Medical and Health sciences > Clinical medicine

Other information

Authenticus ID: P-000-8MR

DOI: 10.1016/j.bcmd.2004.05.003

Abstract (EN): beta2-microglobulin knockout (beta2m(-/-)) mice represent an instructive model of spontaneous iron overload resembling genetic hemochromatosis. The mechanism of iron accumulation in this mouse model may be more complex than involving the MHC class I-like protein HFE. We report that beta2m-deficient mice, like Hfe(-/-) mice, lack the adaptive hepatic hepcidin mRNA increase to iron overload. The inverse correlation of hepatic iron levels and hepcidin mRNA expression in six beta2m(-/-) mice underlines the importance of hepcidin in regulating body iron stores. In contrast to Hfe(-/-) mice, beta2m-deficient mice display increased expression of the duodenal iron transporters DMT1 and ferroportin 1. This result implicates a broader role of beta2m in mammalian iron metabolism, suggesting that (an) additional beta2m-interacting protein(s) could be involved in controlling iron homeostasis, and highlighting the emerging connection of iron metabolism with the immune system.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 7