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Article

Deletion of TMPRSS6 attenuates the phenotype in a mouse model of β-thalassemia.

Blood - 24 May 2012

Nai Antonella, Pagani Alessia, Mandelli Giacomo, Lidonnici Maria Rosa, Silvestri Laura, Ferrari Giuliana, Camaschella Clara

Abstract excerpt

Inappropriately low expression of the key iron regulator hepcidin (HAMP) causes iron overload in untransfused patients affected by β-thalassemia intermedia and Hamp modulation provides improvement of the thalassemic phenotype of the Hbb(th3/+) mouse. HAMP expression is activated by iron through the bone morphogenetic protein (BMP)-son of mothers against decapentaplegic signaling pathway and inhibited by...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 22490684
DOI: 10.1182/blood-2012-01-401885
PMCID: PMC3426375

Genes

HAMP
TMPRSS6

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Deletion of TMPRSS6 attenuates the phenotype in a mouse model of β-thalassemia.PMID 22490684Read ↗

Reducing TMPRSS6 ameliorates hemochromatosis and β-thalassemia in miceSemantic proximity Read ↗

An RNAi therapeutic targeting Tmprss6 decreases iron overload in Hfe−/− mice and ameliorates anemia and iron overload in murine β-thalassemia intermediaSemantic proximity Read ↗

Down-regulation of Bmp/Smad signaling by Tmprss6 is required for maintenance of systemic iron homeostasisSemantic proximity Read ↗

The serine protease TMPRSS6 is required to sense iron deficiency.Semantic proximity Read ↗

Ineffective erythropoiesis in beta-thalassemia is characterized by increased iron absorption mediated by down-regulation of hepcidin and up-regulation of ferroportin.Semantic proximity Read ↗

TMPRSS6 rs855791 modulates hepcidin transcription in vitro and serum hepcidin levels in normal individualsSemantic proximity Read ↗

Severe microcytic anemia but increased erythropoiesis in mice lacking Hfe or Tfr2 and Tmprss6.Semantic proximity Read ↗

Tmprss6 is a genetic modifier of the Hfe-hemochromatosis phenotype in mice.Semantic proximity Read ↗

TMPRSS6 as a Therapeutic Target for Disorders of Erythropoiesis and Iron HomeostasisSemantic proximity Read ↗

Bone morphogenetic proteins as regulators of iron metabolism.Semantic proximity Read ↗

Iron-deficiency anemia secondary to mutations in genes controlling hepcidin.Semantic proximity Read ↗

Role of matriptase-2 (TMPRSS6) in iron metabolism.Semantic proximity Read ↗

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