Gaël NICOLAS (0000-0002-1671-8274)

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In 2001, I described for the first time the critical role of hepcidin in the regulation of iron homeostasis. At that time, I made the demonstration that hepcidin is an hyposideremic hormone acting to repress the iron availability into the body. I also made the demonstration that hepcidin expression is regulated by anemia, hypoxia and inflammation. I described that EPO injections completely turn off hepcidin expression. I demonstrated that hemochromatosis is a metabolic disease due an insufficient production of hepcidin. I made the proof-of-principle that restoring a normal production of hepcidin protects the organism against iron overload. I would like to convince a broad scientific community that all these works had completely changed the view of iron homeostasis, explained many diseases and opened many unexplored new avenues into the field of iron homeostasis and its pathologies. Hepcidin is the iron-regulatory hormone that was searched for more than 40 years.

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French Institute of Health and Medical Research (INSERM), Paris, Government: Paris, 75, FR

2002-10-01 to present | Research Fellow (INSERM U119)

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Gaël NICOLAS

https://orcid.org/0000-0002-1671-8274

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French Institute of Health and Medical Research (INSERM), Paris, Government: Paris, 75, FR

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Search for erythroid regulators involved in iron homeostasis

Works (50 of 76)

"I just wanted more": Hereditary cancer syndromes patients' perspectives on the utility of circulating tumour DNA testing for cancer screening

Assessment of parental mosaicism rates in neurodevelopmental disorders caused by apparent de novo pathogenic variants using deep sequencing

Association of Pick's disease with the <i>MAPT </i>H2 haplotype

Biallelic NAA60 variants with impaired n-terminal acetylation capacity cause autosomal recessive primary familial brain calcifications

Neuronal downregulation of PLCG2 impairs synaptic function and elicits Alzheimer disease hallmarks

Penetrance, variable expressivity and monogenic neurodevelopmental disorders

The detection of a strong episignature for Chung-Jansen syndrome, partially overlapping with Börjeson-Forssman-Lehmann and White-Kernohan syndromes

The microbiota and the host organism switch between cooperation and competition based on dietary iron levels

Whole Exome/Genome Sequencing Joint Analysis of a Family with Oligogenic Familial Hypercholesterolemia

ABCB6 polymorphisms are not overly represented in patients with porphyria

Phlebotomy as an efficient long-term treatment of congenital erythropoietic porphyria

The ubiquitous mitochondrial protein unfoldase CLPX regulates erythroid heme synthesis by control of iron utilization and heme synthesis enzyme activation and turnover

Inherited glycosylphosphatidylinositol defects cause the rare Emm-negative blood phenotype and developmental disorders

αII-spectrin controls calcium-regulated exocytosis in neuroendocrine chromaffin cells through neuronal Wiskott-Aldrich Syndrome protein interaction

Lack of the multidrug transporter MRP4/ABCC4 defines the PEL-negative blood group and impairs platelet aggregation

Erythroid-Progenitor-Targeted Gene Therapy Using Bifunctional TFR1 Ligand-Peptides in Human Erythropoietic Protoporphyria

Regulation and tissue-specific expression of delta-aminolevulinic acid synthases in non-syndromic sideroblastic anemias and porphyrias

Iron deficiency and anemia in adolescent girls consuming predominantly plant-based diets in rural Ethiopia

From a dominant to an oligogenic model of inheritance with environmental modifiers in acute intermittent porphyria

Mutation in human CLPX elevates levels of δ-aminolevulinate synthase and protoporphyrin IX to promote erythropoietic protoporphyria.

A MUTATION IN THE IRON RESPONSE ELEMENT (IRE) OF THE GENE ALAS2 IS A MODIFIER OF CLINICAL GRAVITY IN ERYTHROPOIETIC PROTOPORRHYRIA

The microbiota shifts the iron sensing of intestinal cells.

LC-MS/MS method for hepcidin-25 measurement in human and mouse serum: clinical and research implications in iron disorders.

Porphyrias: A 2015 update.

Matriptase-2 is essential for hepcidin repression during fetal life and postnatal development in mice to maintain iron homeostasis

INCREASED HEPCIDIN MRNA LEVELS AND REDUCTION OF MEMBRANE HEMOJUVELIN IN TMPRSS6-/- PRIMARY HEPATOCYTES

Inactive matriptase-2 mutants found in IRIDA patients still repress hepcidin in a transfection assay despite having lost their serine protease activity

Proximal giant neurofilamentous axonopathy in mice genetically engineered to resist calpain and caspase cleavage of α-II spectrin

1,2-Diacetylbenzene Induces Lou Gehrig Disease-Like Proximal Giant Neurofilamentous Axonopathy in alpha II-Spectrin Calpain-Caspase Resistant Mutant Mice

Iron-deficiency anemia from matriptase-2 inactivation is dependent on the presence of functional Bmp6

Is EPO therapy able to correct iron deficiency anaemia caused by matriptase-2 deficiency?

Hepcidin targets ferroportin for degradation in hepatocytes

A mutant αII-spectrin designed to resist calpain and caspase cleavage questions the functional importance of this process in vivo

Chronic hepcidin induction causes hyposideremia and alters the pattern of cellular iron accumulation in hemochromatotic mice

Spectrin interacts with EVL (Enabled/vasodilator-stimulated phosphoprotein-like protein), a protein involved in actin polymerization

alpha II-Spectrin interacts with Tes and EVL, two actin-binding proteins located at cell contacts

Deciphering the action mechanism of hepcidin | Le mécanisme d'action de l'hepcidine déchiffré

Deciphering the action mechanism of hepcidin,Le mécanisme d'action de l'hepcidine déchiffré

Deregulation of proteins involved in iron metabolism in hepcidin-deficient mice

Iron- and inflammation-induced hepcidin gene expression in mice is not mediated by Kupffer cells in vivo

αII-spectrin interacts with Tes and EVL, two actin-binding proteins located at cell contacts

Functional differences between hepcidin 1 and 2 in transgenic mice

Hepcidin, a candidate modifier of the hemochromatosis phenotype in mice

Mechanisms controlling the regulation of intestinal iron absorption | Mécanismes impliqués dans la régulation de l'absorption intestinale du fer

Mechanisms controlling the regulation of intestinal iron absorption,Mécanismes impliqués dans la régulation de l'absorption intestinale du fer

Transferrin receptor 1 mRNA is downregulated in placenta of hepcidin transgenic embryos

αII-Spectrin is an in vitro target for caspase-2, and its cleavage is regulated by calmodulin binding

Constitutive hepcidin expression prevents iron overload in a mouse model of hemochromatosis

Hepcidin, a key regulator of iron metabolism | L'hepcidine, un régulateur majeur du métabolisme du fer

Hepcidin, a key regulator of iron metabolism,L'hepcidine, un régulateur majeur du métabolisme du fer