AnatomyDiseasesChemicals and DrugsPhenomena and Processes
Cation Transport ProteinsHepcidinsAntimicrobial Cationic PeptidesIronIron Metabolism DisordersIron OverloadHemochromatosisApoferritinsRNA, NuclearFerritinsCeruloplasminEnterocytesDuodenumReceptors, TransferrinIron, DietaryIron-Regulatory ProteinsTransferrinIron Regulatory Protein 2HomeostasisNitrilotriacetic AcidIron Regulatory Protein 1Anemia, Iron-DeficiencyMacrophagesIron-Binding ProteinsCaco-2 CellsMononuclear Phagocyte SystemFerric CompoundsPolycythemiaIntestinal Absorption
SLC40A1FerritinTransmembraneEffluxFpn1Hereditary hemochromatosisMacrophagesDegradationTransferrinGeneTransportersIntestinal Iron AbsorptionELISA KitBindsCellularMutationsEnterocytesDuodenalSystemic ironMRNASmall intestineIntracellularTransporterEncodesReticuloendothelialExpressionLiverEncodeManganeseLevelsDietary ironMutationHumanBacterialZincReceptorSynthesisCellsCytokinesIron exportRarelyBoneMembranesSerumImmune
SLC40A19
  • Ferroportin-1, also known as solute carrier family 40 member 1 (SLC40A1) or iron-regulated transporter 1 (IREG1), is a protein that in humans is encoded by the SLC40A1 gene, and is part of the Ferroportin (Fpn) Family (TC# 2.A.100). (wikipedia.org)
  • Mouse embryos lacking the Slc40a1 gene are aborted before gastrulation occurs, suggesting that the Fpn1 protein encoded is necessary and essential for normal embryonic development. (wikipedia.org)
  • It is known that ferroportin (SLC40A1) gene is expressed at a low level in infertile women. (wikipedia.org)
  • The SLC40A1 gene provides instructions for making a protein called ferroportin. (medlineplus.gov)
  • Mutation c.744G → T (Q248H), located in exon 6 of the ferroportin gene SLC40A1, is found as a polymorphism in populations of African origin. (unl.pt)
  • Hepdicin exerts its biological function upon binding to the only known cellular iron exporter, ferroportin (SLC40A1), thereby leading to ferroportin internalization and degradation. (haematologica.org)
  • utations in the human gene encoding ferroportin 1 (FPN1/IREG-1/MTP-1/SLC40A1) are associated with an unusual iron overload syndrome, recently named hemochromatosis type 4 or ferroportin disease. (intrinsiclifesciences.com)
  • When iron is in excess, hepcidin/HAMP levels increase resulting in a degradation of ferroportin/SLC40A1 limiting the iron efflux to plasma (PubMed:22682227, PubMed:29237594, PubMed:32814342). (icr.ac.uk)
  • It results from an autosomal dominant mutation in the SLC40A1 gene and affects the ability of ferroportin to bind hepcidin. (msdmanuals.com)
Ferritin4
  • If the iron concentration is too low, the IRP concentration increases, thus inhibiting the ferroportin translation and increasing intracellular iron and ferritin concentrations. (wikipedia.org)
  • We also show in FPN1-deficient primary macrophages derived from patients with ferroportin disease that hinokitiol moves labile iron from inside to outside cells and decreases intracellular ferritin levels. (illinois.edu)
  • There are several possible fates for endocytosed ferritin including catabolism of the protein in lysosomes, excretion in the bile or inclusion in the endogenous ferritin pool. (diff.org)
  • The proteins hepcidin, ferroportin, transferrin receptors, and ferritin are essential for iron metabolism . (powerofpositivity.com)
Transmembrane6
  • Ferroportin is a transmembrane protein that transports iron from the inside of a cell to the outside of the cell. (wikipedia.org)
  • Members of the ferroportin family consist of 400-800 amino acid residues, with a highly conserved histidine at residue position 32 (H32), and exhibit 8-12 putative transmembrane domains. (wikipedia.org)
  • Ferroportin is a transmembrane protein responsible for iron export from enterocytes and macrophages. (unl.pt)
  • Deficiencies of the transmembrane iron-transporting protein ferroportin (FPN1) cause the iron misdistribution that underlies ferroportin disease, anemia of inflammation, and several other human diseases and conditions. (illinois.edu)
  • A small molecule natural product, hinokitiol, was recently shown to serve as a surrogate transmembrane iron transporter that can restore hemoglobinization in zebrafish deficient in other iron transporting proteins and can increase gut iron absorption in FPN1-deficient flatiron mice. (illinois.edu)
  • As the master regulator of iron homeostasis in the body, hepcidin acts by binding and degrading the transmembrane protein ferroportin to keep it from releasing too much iron into the bloodstream. (jillcarnahan.com)
Efflux2
  • Intracellular iron levels are elaborately balanced by iron efflux, uptake, and storage proteins that are regulated by iron regulatory proteins (IRPs, including IRP1 and IRP2) [ 16 ]. (nature.com)
  • Hepcidin inhibits the cellular efflux of iron by binding to and inducing the degradation of ferroportin (FPN) , the sole iron exporter in iron-transporting cells, thereby blocking iron flow into plasma. (diff.org)
Fpn11
Hereditary hemochromatosis3
  • Patients with mutant ferroportin proteins that do not localize to the cell surface show typical ferroportin disease with low transferrin saturation and early Küpffer cell iron loading, while patients with mutant proteins unable to respond to hepcidin show high transferrin saturation and early hepatocyte iron loading similar to classic hereditary hemochromatosis. (unimol.it)
  • Type 2 hereditary hemochromatosis (juvenile hemochromatosis) is a rare autosomal recessive disorder caused by mutations in the HJV gene that affect the transcription protein hemojuvelin, or mutations in the HAMP gene, which directly codes for hepcidin. (msdmanuals.com)
  • Type 4 hereditary hemochromatosis (ferroportin disease) occurs largely in people of southern European ancestry. (msdmanuals.com)
Macrophages6
  • Ferroportin is found on the basolateral membranes of intestinal epithelia of mammals, including: Enterocytes in the duodenum Hepatocytes Macrophages of the reticuloendothelial system Adipocytes Ferroportin-1 plays an important role in neural tube closure and forebrain patterning. (wikipedia.org)
  • 1 , 7 In addition, pro-and anti-inflammatory cytokines further contribute to iron retention within monocytes/macrophages by stimulating iron uptake pathways while inhibiting ferroportin transcription in these cells. (haematologica.org)
  • Considering the tissue distribution of ferroportin, the hepcidin-ferroportin interaction explains the efferent arm of iron homeostasis because it can control the iron absorption in the duodenum, as well as the release of iron from macrophages and hepatocytes. (diff.org)
  • Ferroportin disease (hemochromatosis type 4) is a recently recognized disorder of human iron metabolism, characterized by iron deposition in macrophages, including Kupffer cells. (intrinsiclifesciences.com)
  • In patients with most, but not all, ferroportin mutations, retention of iron in macrophages of the liver and other organs may protect against damage to parenchymal cells. (intrinsiclifesciences.com)
  • Hepcidin, an iron regulatory protein, is produced by hepatocytes and macrophages in response to proinflammatory stimuli. (biomedcentral.com)
Degradation2
  • Within the phagocytes, iron is recycled through enzymatic degradation of heme and delivered via ferroportin into the circulation, accounting for approximately 90% of the daily iron needs. (haematologica.org)
  • Hepcidin, which is expressed in the liver, heart, 16 and kidney, 17 is the key mediator of anemia of inflammation, 18,19 and synthetic hepcidin was shown to interact physically with ferroportin in a cellular overexpression system, causing internalization and degradation, and decreased export of iron. (intrinsiclifesciences.com)
Transferrin3
  • Lactoferrin is an iron-binding protein similar to transferrin which is present mainly in milk. (diff.org)
  • Iron overload disease due to mutations in ferroportin has a dominant inheritance and a variable clinical phenotype, such that some patients show early Küpffer cell iron loading and low transferrin saturation, while others show hepatocyte iron loading and high transferrin saturation. (unimol.it)
  • Mutations in transferrin receptor 2 ( TFR2 ) gene that codes for a protein that appears to control saturation of transferrin, can cause a rare autosomal recessive form of hemochromatosis. (msdmanuals.com)
Gene5
  • The HFE protein is thought to play an important role in the process by which duodenal crypt cells sense body iron stores, and the variant gene leads to increased iron absorption from the duodenum. (mhmedical.com)
  • An uncommon juvenile-onset variant that is characterized by severe iron overload, cardiac dysfunction, hypogonadotropic hypogonadism, and a high mortality rate is usually linked to a variant gene on chromosome 1q designated HJV that produces a protein called hemojuvelin (type 2a) or, rarely, to a variant of the HAMP gene on chromosome 19 that encodes hepcidin (type 2b). (mhmedical.com)
  • The HFE gene encodes the HFE protein, which regulates the production of hepcidin. (jillcarnahan.com)
  • Mutations in the gene encoding ferroportin 1, a cellular iron exporter, are responsible for this iron storage disease, inherited as an autosomal dominant trait. (intrinsiclifesciences.com)
  • Our research may help us better predict which tissues and cells new viruses will infect and also provides a potential therapeutic target to regulate tissue-specific gene expression without changing the translated protein," said Miller. (rna-seqblog.com)
Transporters6
  • Recent crystal structures generated from a bacterial homologue of ferroportin (from Bdellovibrio bacteriovorus) revealed that the Fpn structure resembles that of major facilitator superfamily (MFS) transporters. (wikipedia.org)
  • The prospective substrate binding site is located at the interface between the N-terminal and C-terminal halves of the protein, and is alternately accessible from either side of the cell membrane, consistent with MFS transporters. (wikipedia.org)
  • Here, we have analyzed where messenger RNAs that encode iron transport proteins are expressed in the brain, using the Allen Brain atlas, and we conclude that several important iron transporters are highly expressed in the choroid plexus. (elsevierpure.com)
  • Iron supplements taken daily at doses of 60mg have consistently been shown to increase hepcidin levels, which shuts down the ferroportin transporters and iron uptake into the blood. (drwholeness.com)
  • If too much iron is being absorbed more hepcidin is created, leading to more destruction of ferroportin transporters. (drwholeness.com)
  • These cells contain proteins known as iron transporters, such as divalent metal transporter 1 (DMT1), which facilitate the uptake of ferrous iron into the enterocytes. (zeroharm.in)
Intestinal Iron Absorption1
  • It is suggested that hepcidin also decreases expression of proteins involved in intestinal iron absorption, such as duodenal cytochrome-b ( Dcytb ) and divalent metal transporter-1 ( DMT1 ), although the mechanism and extent of control is unknown. (diff.org)
ELISA Kit1
  • Human Ferroportin (FPN) ELISA Kit - 5x 96 wells plate - 1 kit is backordered and will ship as soon as it is back in stock. (dnamethsoc.com)
Binds1
  • Ferroportin is inhibited by hepcidin, which binds to ferroportin and internalizes it within the cell. (wikipedia.org)
Cellular2
  • Moreover, modulation of the proteins involved in cellular metal homeostasis may help in the regulation of metabolism, adaptability to a diverse range of environmental conditions, and biofortification. (frontiersin.org)
  • The etiology of Parkinson's disease (PD) is linked with cellular inclusions in the substantia nigra pars compacta region of the brain that are enriched in the misfolded presynaptic protein α -synuclein ( α S) and death of the dopaminergic neurons. (hindawi.com)
Mutations1
  • The C282Y/C282Y mutations (homozygosity for C282Y ) lead to decreased synthesis of the iron hormone hepcidin, which in turn causes an increased activity of the iron export protein ferroportin both at the digestive and splenic levels. (wjgnet.com)
Enterocytes1
Duodenal2
  • In mice treated with omeprazole, protein levels of duodenal and splenic ferroportin decreased. (tokushima-u.ac.jp)
  • Taken together, PPIs directly affect iron metabolism by suppressing iron absorption through the inhibition of duodenal ferroportin via hepcidin upregulation. (tokushima-u.ac.jp)
Systemic iron2
  • Hepcidin is a liver-derived peptide that oversees systemic iron changes by determining iron absorption and iron release from the cells through binding to its receptor, the iron-export protein ferroportin. (uni-ulm.de)
  • In conclusion, the systemic iron burden in ferroportin disease is not a sufficient cause for chronic liver disease. (intrinsiclifesciences.com)
MRNA2
  • It is also important to mention that, not only is ferroportin down-regulated in granulosa cells, but also in cervical cells of infertile women, and that the association between infertility and low ferroportin levels in these cells can be seen, again, when mRNA ferroportin levels was adjusted by age and smoking status. (wikipedia.org)
  • The hepcidin mRNA lacks any stem-loop structures containing the consensus IRE motif for binding of iron-regulatory proteins. (diff.org)
Small intestine2
  • After dietary iron is absorbed into the cells of the small intestine, ferroportin allows that iron to be transported out of those cells and into the bloodstream. (wikipedia.org)
  • Ferroportin then transports iron from the small intestine into the bloodstream, and the iron is carried by the blood to the tissues and organs of the body. (medlineplus.gov)
Intracellular1
  • HJV is proposed to act as a co-receptor for bone morphogenic protein ( BMP ) ligands and BMP receptors (BMP-R). Upon binding to HJV, the ligand and two BMPR s on the cell surface, the intracellular BMP signaling pathway is activated. (diff.org)
Transporter1
  • Hepcidin is created by the liver and will destroy the transporter protein, ferroportin if there is too much iron showing up at the enterocyte. (drwholeness.com)
Encodes1
Reticuloendothelial1
  • Ferroportin also transports iron out of reticuloendothelial cells in the liver, spleen, and bone marrow. (medlineplus.gov)
Expression4
  • What's more, low expression of ferroportin is also associated with infertility when some features like age and smoking habits are considered. (wikipedia.org)
  • Ferroportin expression is also regulated by the IRP regulatory mechanism. (wikipedia.org)
  • DMT1 appears to be regulated by iron levels with protein expression increased in iron loaded liver, lower in control liver, and not detected in iron deficient livers. (diff.org)
  • It is suggested that HJV and soluble HJV (sHJV) modulates hepcidin expression through activating bone morphogenetic protein (BMP) signaling. (diff.org)
Liver1
  • The increased levels of this protein in the serum may be due to a variety of physiological situations or previously diagnosed comorbidities that may be directly or indirectly related to liver dysfunction. (scielo.br)
Encode1
  • The research team says this work is important because while there are multiple ways for our RNA to encode the same proteins, the specific RNA sequence is important to regulate protein and RNA levels. (rna-seqblog.com)
Manganese1
  • Ferroportin may also function as a manganese exporter. (wikipedia.org)
Levels2
Dietary iron1
  • Structural studies of human ferroportin (King's College London)Ferroportin is a protein that is involved in transferring dietary iron from intestines to blood. (ukri.org)
Mutation1
  • We present clinical, histopathological, and radiological findings in a family with the most common ferroportin mutation, V162del. (intrinsiclifesciences.com)
Human5
  • The MSDS of Human for Ferroportin is available from Karlan upon request. (dnamethsoc.com)
  • Human IgG antibody Laboratories manufactures the ferroportin antibody rat reagents distributed by Genprice. (exoquick.com)
  • The Ferroportin Antibody Rat reagent is RUO (Research Use Only) to test human serum or cell culture lab samples. (exoquick.com)
  • Moreover, iron interactions with N-terminally acetylated α S, the physiologically relevant form of the human protein, will be addressed to shed light on the current understanding of protein dynamics and the physiological environment in the disease state. (hindawi.com)
  • A tetrameric form of α S has also been identified in human tissues that has been postulated as the inactive storage form of this structurally dynamic protein due to its aggregation resistance [ 6 , 7 ]. (hindawi.com)
Bacterial1
  • Studies of bacterial protein-DNA complexes (Portsmouth)All bacterial species have an 'immune system', whereby their own DNA can be distinguished from foreign DNA. (ukri.org)
Zinc2
  • In addition to iron, ferroportin has been shown to transport cobalt & zinc, as well as nickel. (wikipedia.org)
  • Both proteins contain a Cysteine-rich zinc finger domain that is highly specific to DnaJ-like molecular chaperons. (yokohama-cu.ac.jp)
Receptor1
  • The first is low-density lipoprotein receptor-related protein (LRP) and the second is the major ( RHL -1 ) subunit of the asialoglycoprotein receptor. (diff.org)
Synthesis1
Cells1
  • Iron accumulates in cells that lack plasma membrane ferroportin, which leads to lower amounts of circulating iron available for erythropoiesis. (biomedcentral.com)
Cytokines1
  • The changes in body iron distribution are caused by cytokines and acute phase proteins. (haematologica.org)
Iron export1
  • HSC express the iron-uptake protein TfR1, and the iron-export protein ferroportin. (westminster.ac.uk)
Rarely1
  • I have given 60mg of elemental iron using hydrolzyed whole protein chelated iron in a few cases but rarely is it necessary even in the most deficient cases. (drwholeness.com)
Bone1
  • The bone morphogenetic proteins (BMPs) are a family of secreted signaling molecules that can induce ectopic bone growth. (antibodies-online.com)
Membranes2
  • It may also improve your iron metabolism as some of its sulphur compounds increase production of a protein called ferroportin, which carries iron across cell membranes. (viva.org.uk)
  • NMR/neutron studies of a mechanosensitive channel protein (Oxford) We will study a 'mechanosensitive' protein from membranes that controls cell shape and size in different environments. (ukri.org)
Serum1
  • Description: A competitive ELISA for quantitative measurement of Rat Ferroportin in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. (exoquick.com)
Immune1