Hervé Puy
Co-Responsable de l'équipe, Hème, fer et pathologies inflammatoires
Institution:
USPC - SET - SVS
Summary
Published articles Show More
Heterozygous Mutations in BMP6 Pro-peptide Lead to Inappropriate Hepcidin Synthesis and Moderate Iron Overload in Humans...
...Published in Gastroenterology
Background & Aims Hereditary hemochromatosis is a heterogeneous group of genetic disorders characterized by parenchymal iron overload. It is caused by defective expression of liver hepcidin, the main regulator of iron homeostasis. Iron stimulates the gene encoding (HAMP) hepcidin via the BMP6 signaling to SMAD. Although several genetic factors have...
Hepcidin as a Major Component of Renal Antibacterial Defenses against Uropathogenic Escherichia coli
...Published in Methods Mol Biol
The iron-regulatory peptide hepcidin exhibits antimicrobial activity. Having previously shown hepcidin expression in the kidney, we addressed its role in urinary tract infection (UTI), which remains largely unknown. Experimental UTI was induced in wild-type (WT) and hepcidin-knockout (Hepc−/−) mice using the uropathogenic Escherichia coli CFT073 st...
Iron regulatory protein 1 sustains mitochondrial iron loading and function in frataxin deficiency
...
Published in Cell Metabolism
Mitochondrial iron accumulation is a hallmark of diseases associated with impaired iron-sulfur cluster (Fe-S) biogenesis, such as Friedreich ataxia linked to frataxin (FXN) deficiency. The pathophysiological relevance of the mitochondrial iron loading and the underlying mechanisms are unknown. Using a mouse model of hepatic FXN deficiency in combin...
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Experience
Co-Responsable de l'équipe
Université Paris Diderot
Hème, fer et pathologies inflammatoires; Département Néphrologie, Immunologie et Hématologie (NIH); UMR 1149, Centre de Recherche sur l'Inflamation (CRI); UFR de Médecine
