RGD Reference Report - mRNA expression of iron regulatory genes in beta-thalassemia intermedia and beta-thalassemia major mouse models. - Rat Genome Database

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mRNA expression of iron regulatory genes in beta-thalassemia intermedia and beta-thalassemia major mouse models.

Authors: Weizer-Stern, O  Adamsky, K  Amariglio, N  Rachmilewitz, E  Breda, L  Rivella, S  Rechavi, G 
Citation: Weizer-Stern O, etal., Am J Hematol. 2006 Jul;81(7):479-83.
RGD ID: 11062138
Pubmed: PMID:16755567   (View Abstract at PubMed)
DOI: DOI:10.1002/ajh.20549   (Journal Full-text)

beta-Thalassemia is an inherited anemia in which synthesis of the hemoglobin beta-chain is decreased. The excess unmatched alpha-globin chains accumulate in the growing erythroid precursors, causing their premature death (ineffective erythropoiesis). Clinical features of beta-thalassemia include variably severe anemia and iron accumulation due to increased intestinal iron absorption. The most anemic patients require regular blood transfusions, which exacerbate their iron overload and result in damage to vital organs. The hepatic peptide hepcidin, a key regulator of iron metabolism in mammals, was recently found to be low in the urine of beta-thalassemia patients, compared with healthy controls, despite their iron overload. In our work, we measured by RQ-PCR the liver mRNA expression of hepcidin and other iron regulatory genes in beta-thalassemia major mouse model (C57Bl/6 Hbb(th3/th3)), and compared it with beta-thalassemia intermedia mouse model (C57Bl/6 Hbb(th3/+)) and control mice. We found decreased expression of hepcidin and TfR2 and increased expression of TfR1 and NGAL in the beta-thalassemia mouse models, compared with the control mice. Significant down-regulation of hepcidin expression in beta-thalassemia major, despite iron overload, might explain the increased iron absorption typically observed in thalassemia.




  
Object Symbol
Species
Term
Qualifier
Evidence
With
Notes
Source
Original Reference(s)
TFR2Humanbeta thalassemia  ISORGD:1320888mRNA:decreased expression:liver:RGD 
TFRCHumanbeta thalassemia  ISORGD:70503mRNA:increased expression:liver:RGD 
Tfr2Ratbeta thalassemia  ISORGD:1320888mRNA:decreased expression:liver:RGD 
Tfr2Mousebeta thalassemia  IEP mRNA:decreased expression:liver:RGD 
TfrcRatbeta thalassemia  ISORGD:70503mRNA:increased expression:liver:RGD 
TfrcMousebeta thalassemia  IEP mRNA:increased expression:liver:RGD 


Genes (Rattus norvegicus)
Tfr2  (transferrin receptor 2) Tfrc  (transferrin receptor)

Genes (Mus musculus)
Tfr2  (transferrin receptor 2) Tfrc  (transferrin receptor)

Genes (Homo sapiens)
TFR2  (transferrin receptor 2) TFRC  (transferrin receptor)