Published in Am J Hematol on April 01, 2015
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Combination of Tmprss6- ASO and the iron chelator deferiprone improves erythropoiesis and reduces iron overload in a mouse model of beta-thalassemia intermedia. Haematologica (2015) 0.81
Modulation of hepcidin to treat iron deregulation: potential clinical applications. Expert Rev Hematol (2015) 0.79
Retinal abnormalities in β-thalassemia major. Surv Ophthalmol (2015) 0.77
Hepcidin: A Promising Therapeutic Target for Iron Disorders: A Systematic Review. Medicine (Baltimore) (2016) 0.76
New strategies to target iron metabolism for the treatment of beta thalassemia. Ann N Y Acad Sci (2016) 0.75
Minihepcidin peptides as disease modifiers in mice affected by β-thalassemia and polycythemia vera. Blood (2016) 0.75
3,1-Benzothiazines, 1,4-Benzodioxines and 1,4-Benzoxazines as Inhibitors of Matriptase-2: Outcome of a Focused Screening Approach. Pharmaceuticals (Basel) (2016) 0.75
Decreasing TfR1 expression reverses anemia and hepcidin suppression in β-thalassemic mice. Blood (2017) 0.75
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The serine protease matriptase-2 (TMPRSS6) inhibits hepcidin activation by cleaving membrane hemojuvelin. Cell Metab (2008) 3.78
Hepcidin in iron overload disorders. Blood (2005) 2.88
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Hepcidin as a therapeutic tool to limit iron overload and improve anemia in β-thalassemic mice. J Clin Invest (2010) 2.18
Transferrin therapy ameliorates disease in beta-thalassemic mice. Nat Med (2010) 2.04
Transfusion suppresses erythropoiesis and increases hepcidin in adult patients with β-thalassemia major: a longitudinal study. Blood (2013) 2.03
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An RNAi therapeutic targeting Tmprss6 decreases iron overload in Hfe(-/-) mice and ameliorates anemia and iron overload in murine β-thalassemia intermedia. Blood (2012) 1.94
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Reducing TMPRSS6 ameliorates hemochromatosis and β-thalassemia in mice. J Clin Invest (2013) 1.44
Liver expression of hepcidin and other iron genes in two mouse models of beta-thalassemia. Haematologica (2006) 1.34
Deletion of TMPRSS6 attenuates the phenotype in a mouse model of β-thalassemia. Blood (2012) 1.25
mRNA expression of iron regulatory genes in beta-thalassemia intermedia and beta-thalassemia major mouse models. Am J Hematol (2006) 1.22
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The M2 splice isoform of pyruvate kinase is important for cancer metabolism and tumour growth. Nature (2008) 18.20
From noncoding variant to phenotype via SORT1 at the 1p13 cholesterol locus. Nature (2010) 12.07
Regulation of progenitor cell proliferation and granulocyte function by microRNA-223. Nature (2008) 8.69
Immunoassay for human serum hepcidin. Blood (2008) 5.32
Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood (2010) 4.96
Therapeutic RNAi targeting PCSK9 acutely lowers plasma cholesterol in rodents and LDL cholesterol in nonhuman primates. Proc Natl Acad Sci U S A (2008) 4.57
Lipid-like materials for low-dose, in vivo gene silencing. Proc Natl Acad Sci U S A (2010) 4.25
Common variants at 10 genomic loci influence hemoglobin A₁(C) levels via glycemic and nonglycemic pathways. Diabetes (2010) 4.07
Mutations in TMPRSS6 cause iron-refractory iron deficiency anemia (IRIDA). Nat Genet (2008) 4.02
Safety and efficacy of RNAi therapy for transthyretin amyloidosis. N Engl J Med (2013) 3.97
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Immortalization and transformation of primary human airway epithelial cells by gene transfer. Oncogene (2002) 3.80
Identification of a ferrireductase required for efficient transferrin-dependent iron uptake in erythroid cells. Nat Genet (2005) 3.28
Targeted delivery of RNAi therapeutics with endogenous and exogenous ligand-based mechanisms. Mol Ther (2010) 3.26
A mouse model of juvenile hemochromatosis. J Clin Invest (2005) 3.25
A mutation in the TMPRSS6 gene, encoding a transmembrane serine protease that suppresses hepcidin production, in familial iron deficiency anemia refractory to oral iron. Haematologica (2008) 2.92
Image-based analysis of lipid nanoparticle-mediated siRNA delivery, intracellular trafficking and endosomal escape. Nat Biotechnol (2013) 2.81
Hepcidin--a potential novel biomarker for iron status in chronic kidney disease. Clin J Am Soc Nephrol (2009) 2.61
Highly penetrant, rapid tumorigenesis through conditional inversion of the tumor suppressor gene Snf5. Cancer Cell (2002) 2.51
The Steap proteins are metalloreductases. Blood (2006) 2.37
Mutations in mitochondrial carrier family gene SLC25A38 cause nonsyndromic autosomal recessive congenital sideroblastic anemia. Nat Genet (2009) 2.37
Cdk5rap2 regulates centrosome function and chromosome segregation in neuronal progenitors. Development (2010) 2.31
Down-regulation of Bmp/Smad signaling by Tmprss6 is required for maintenance of systemic iron homeostasis. Blood (2010) 2.20
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Haem homeostasis is regulated by the conserved and concerted functions of HRG-1 proteins. Nature (2008) 1.95
An RNAi therapeutic targeting Tmprss6 decreases iron overload in Hfe(-/-) mice and ameliorates anemia and iron overload in murine β-thalassemia intermedia. Blood (2012) 1.94
Silencing of lipid metabolism genes through IRE1α-mediated mRNA decay lowers plasma lipids in mice. Cell Metab (2012) 1.90
Endogenous oncogenic Nras mutation promotes aberrant GM-CSF signaling in granulocytic/monocytic precursors in a murine model of chronic myelomonocytic leukemia. Blood (2010) 1.86
T-lymphoblastic lymphoma cells express high levels of BCL2, S1P1, and ICAM1, leading to a blockade of tumor cell intravasation. Cancer Cell (2010) 1.85
The Scap/SREBP pathway is essential for developing diabetic fatty liver and carbohydrate-induced hypertriglyceridemia in animals. Cell Metab (2012) 1.79
Serum hepcidin is significantly associated with iron absorption from food and supplemental sources in healthy young women. Am J Clin Nutr (2008) 1.79
Treatment of erythropoietin deficiency in mice with systemically administered siRNA. Blood (2012) 1.64
Erythropoietin stimulates phosphorylation and activation of GATA-1 via the PI3-kinase/AKT signaling pathway. Blood (2005) 1.63
Testosterone suppresses hepcidin in men: a potential mechanism for testosterone-induced erythrocytosis. J Clin Endocrinol Metab (2010) 1.50
Perturbation of hepcidin expression by BMP type I receptor deletion induces iron overload in mice. Blood (2011) 1.49
The mitochondrial ATP-binding cassette transporter Abcb7 is essential in mice and participates in cytosolic iron-sulfur cluster biogenesis. Hum Mol Genet (2006) 1.47
Iron-responsive degradation of iron-regulatory protein 1 does not require the Fe-S cluster. EMBO J (2006) 1.43
Systematic molecular genetic analysis of congenital sideroblastic anemia: evidence for genetic heterogeneity and identification of novel mutations. Pediatr Blood Cancer (2010) 1.43
Transferrin is a major determinant of hepcidin expression in hypotransferrinemic mice. Blood (2010) 1.41
X-linked gray platelet syndrome due to a GATA1 Arg216Gln mutation. Blood (2007) 1.38
nm1054: a spontaneous, recessive, hypochromic, microcytic anemia mutation in the mouse. Blood (2005) 1.38
Modulation of hepcidin production during hypoxia-induced erythropoiesis in humans in vivo: data from the HIGHCARE project. Blood (2010) 1.37
Two nonsense mutations in the TMPRSS6 gene in a patient with microcytic anemia and iron deficiency. Blood (2008) 1.35
Multivalent N-acetylgalactosamine-conjugated siRNA localizes in hepatocytes and elicits robust RNAi-mediated gene silencing. J Am Chem Soc (2014) 1.31
IRE1α activation protects mice against acetaminophen-induced hepatotoxicity. J Exp Med (2012) 1.30
Lipopeptide nanoparticles for potent and selective siRNA delivery in rodents and nonhuman primates. Proc Natl Acad Sci U S A (2014) 1.30
Monocyte-directed RNAi targeting CCR2 improves infarct healing in atherosclerosis-prone mice. Circulation (2013) 1.30
Abcb7, the gene responsible for X-linked sideroblastic anemia with ataxia, is essential for hematopoiesis. Blood (2006) 1.28
Activation of ER stress and mTORC1 suppresses hepatic sortilin-1 levels in obese mice. J Clin Invest (2012) 1.28
Heme-regulated eIF2alpha kinase modifies the phenotypic severity of murine models of erythropoietic protoporphyria and beta-thalassemia. J Clin Invest (2005) 1.26
Primary ciliary dyskinesia in mice lacking the novel ciliary protein Pcdp1. Mol Cell Biol (2007) 1.26
AKT induces erythroid-cell maturation of JAK2-deficient fetal liver progenitor cells and is required for Epo regulation of erythroid-cell differentiation. Blood (2005) 1.25
Genetic variation in Mon1a affects protein trafficking and modifies macrophage iron loading in mice. Nat Genet (2007) 1.23
SOD2-deficiency anemia: protein oxidation and altered protein expression reveal targets of damage, stress response, and antioxidant responsiveness. Blood (2004) 1.23
Serum hepcidin as a diagnostic test of iron deficiency in premenopausal female blood donors. Haematologica (2011) 1.21
Dynamics of equilibrium structural fluctuations of apomyoglobin measured by fluorescence correlation spectroscopy. Proc Natl Acad Sci U S A (2007) 1.18
A mutation in Sec15l1 causes anemia in hemoglobin deficit (hbd) mice. Nat Genet (2005) 1.16
Cybrd1 (duodenal cytochrome b) is not necessary for dietary iron absorption in mice. Blood (2005) 1.16
Immunosurveillance and survivin-specific T-cell immunity in children with high-risk neuroblastoma. J Clin Oncol (2006) 1.15
Crystal structure of Spot 14, a modulator of fatty acid synthesis. Proc Natl Acad Sci U S A (2010) 1.15
Chemical genetics identifies Rab geranylgeranyl transferase as an apoptotic target of farnesyl transferase inhibitors. Cancer Cell (2005) 1.15
Modular enzyme design: regulation by mutually exclusive protein folding. J Mol Biol (2006) 1.14
The IRP1-HIF-2α axis coordinates iron and oxygen sensing with erythropoiesis and iron absorption. Cell Metab (2013) 1.14
Iron overload in patients with acute leukemia or MDS undergoing myeloablative stem cell transplantation. Biol Blood Marrow Transplant (2010) 1.13
Induced pluripotent stem cells with a mitochondrial DNA deletion. Stem Cells (2013) 1.13
Maternal serum hepcidin is low at term and independent of cord blood iron status. Eur J Haematol (2010) 1.12
Polymeric nanoparticle PET/MR imaging allows macrophage detection in atherosclerotic plaques. Circ Res (2013) 1.11
Biodegradable lipids enabling rapidly eliminated lipid nanoparticles for systemic delivery of RNAi therapeutics. Mol Ther (2013) 1.11
HRG1 is essential for heme transport from the phagolysosome of macrophages during erythrophagocytosis. Cell Metab (2013) 1.09
Mitochondrial Atpif1 regulates haem synthesis in developing erythroblasts. Nature (2012) 1.09
Demonstrating Bioequivalence of Locally Acting Orally Inhaled Drug Products (OIPs): Workshop Summary Report. J Aerosol Med Pulm Drug Deliv (2010) 1.08
Reduction of serum hepcidin by hemodialysis in pediatric and adult patients. Clin J Am Soc Nephrol (2010) 1.07
A time course of hepcidin response to iron challenge in patients with HFE and TFR2 hemochromatosis. Haematologica (2010) 1.07
Identification of a Steap3 endosomal targeting motif essential for normal iron metabolism. Blood (2008) 1.05
Structure of the membrane proximal oxidoreductase domain of human Steap3, the dominant ferrireductase of the erythroid transferrin cycle. Proc Natl Acad Sci U S A (2008) 1.04
Antiproliferative small-molecule inhibitors of transcription factor LSF reveal oncogene addiction to LSF in hepatocellular carcinoma. Proc Natl Acad Sci U S A (2012) 1.04
Harnessing a physiologic mechanism for siRNA delivery with mimetic lipoprotein particles. Mol Ther (2012) 1.04
CD4+/CD56+ hematodermic neoplasm ("blastic natural killer cell lymphoma"): neoplastic cells express the immature dendritic cell marker BDCA-2 and produce interferon. Am J Clin Pathol (2007) 1.03
Bacteriophage P22 tail accessory factor GP26 is a long triple-stranded coiled-coil. J Biol Chem (2004) 1.03
Suppression of iron-regulatory hepcidin by vitamin D. J Am Soc Nephrol (2013) 1.03
Hepcidin induction by transgenic overexpression of Hfe does not require the Hfe cytoplasmic tail, but does require hemojuvelin. Blood (2010) 1.03
A novel syndrome of congenital sideroblastic anemia, B-cell immunodeficiency, periodic fevers, and developmental delay (SIFD). Blood (2013) 1.03
Liver as a target for oligonucleotide therapeutics. J Hepatol (2013) 1.02
Folding of tetrameric p53: oligomerization and tumorigenic mutations induce misfolding and loss of function. J Mol Biol (2009) 1.00
Impact of hemochromatosis gene mutations on cardiac status in doxorubicin-treated survivors of childhood high-risk leukemia. Cancer (2013) 0.99
A rapid two-step method for isolation of functional primary mouse hepatocytes: cell characterization and asialoglycoprotein receptor based assay development. Cytotechnology (2011) 0.99
In vivo reduction of erythrocyte oxidant stress in a murine model of beta-thalassemia. Haematologica (2004) 0.98
Emi1 maintains genomic integrity during zebrafish embryogenesis and cooperates with p53 in tumor suppression. Mol Cell Biol (2009) 0.97
Male infertility and thiamine-dependent erythroid hypoplasia in mice lacking thiamine transporter Slc19a2. Mol Genet Metab (2003) 0.97
Iron homeostasis during cystic fibrosis pulmonary exacerbation. Clin Transl Sci (2012) 0.97
Greater increase in urinary hepcidin predicts protection from acute kidney injury after cardiopulmonary bypass. Nephrol Dial Transplant (2011) 0.95
Heme transport and erythropoiesis. Curr Opin Chem Biol (2013) 0.94
Femoral artery calcification as a determinant of success for percutaneous access for endovascular abdominal aortic aneurysm repair. J Vasc Surg (2013) 0.93
Genome-wide association study follow-up identifies cyclin A2 as a regulator of the transition through cytokinesis during terminal erythropoiesis. Am J Hematol (2015) 0.93
Iron absorption in dysmetabolic iron overload syndrome is decreased and correlates with increased plasma hepcidin. J Hepatol (2009) 0.92