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Iron Metabolism/Iron Disorders

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Question
Answer
Primary storage location of iron in the body   show
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Components and function of Ferritin   show
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Components and function of Hemosiderin   show
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Ferritin not combined with iron   show
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show True.  
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show A iron transport molecule that binds 2 Fe atoms.  
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show (1) Liver (2) Macrophages  
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show Ferrous oxide (Fe+++)  
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show Transferrin receptor binds 2 transferrins. The receptor-tranferrin-iron complex is endocytosed. The endocytotic vacuole fuses with a lysozyme, where at an acid pH, tranferrin releases iron as ferrous form. The iron is transported to mitochondria.  
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Dietary forms of iron   show
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(T or F) Heme iron (animal source) is better absorbed than non-heme iron (vegetable source).   show
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Portion of the intestine that absorps iron   show
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How is dietary heme transported across an enterocyte?   show
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show Heme oxygenase  
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How is non-heme iron or ferric iron transported across an enterocyte?   show
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How does absorbed dietary iron get transported across the basolateral membrane of an enterocyte?   show
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Where is hepcidin manufactred?   show
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Function of hepcidin   show
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How does low plasma iron affect hepcidin synthesis and iron homeostasis?   show
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How does high plasma iron affect hepcidin synthesis and iron homeostasis?   show
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Enzyme found in macrophages that catabolize heme and release ferrous iron from phagocytized erythrocytes   show
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show Ceruloplasmin  
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show Phagocytosis of aged erythrocytes and flawed immature red cells accounts for almost all of the sotrage iron found in the macrophages of the liver, bone marrow, and spleen.  
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show Begins in the mitochondria with elemental sulphur, the cofactor pryridoxal-5-phosphate and a scaffold protein. 2 iron atoms are added to the [Fe-S] cluster and form a single [2Fe-2S] cluster. 2 [2Fe-2S] clusters form a [4Fe-4S]  
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show Iron Regulatory Proteins (IRP-1 and IRP-2) are cytoplasmic RNA binding [Fe-S] cluster proteins. They control translation of ferritin and transferrin receptor synthesis by binding to iron-responsive elements (IRE) in RNA.  
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What is the difference between IRP-1 and IRP-2 (iron regulatory proteins)?   show
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Describe the effects of IRP-1 (iron regulatory proteins) on transferrin receptor (TfR1) syntehsis in the setting of high cellular iron.   show
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show High cellular iron allows [4Fe-4S] cluster formation in IRP-1 (low affinity) which binds to the 5'-IRE of ferritin mRNA, allowing translation into ferritin.  
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Describe the effects of IRP-1 (iron regulatory proteins) on transferrin receptor (TfR1) syntehsis in the setting of low cellular iron.   show
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Describe the effects of IRP-1 (iron regulatory proteins) on ferritin syntehsis in the setting of low cellular iron.   show
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show (1) Pregnancy (2) Periods of rapid growth (3) Blood loss due to heavy menstural bleeding (4) Blood loss due to internal bleeding  
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Signs and symptoms of iron deficiency anemia   show
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show Iron deficiency anemia  
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show Iron deficiency anemia  
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What is the measurement of all iron bound to tranferrin in the body?   show
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show Total iron binding capacity (TIBC)  
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What approximates the amount of tranferrin in the body?   show
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show Serum iron (SI) divided by the Total iron binding capacity (TIBC)  
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show 25-45%  
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What is the soluble transferrin receptor?   show
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MCV and MCHC of microcytic and hypochromic anemia   show
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show Inadequate synthesis of hemoglobin  
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show Anemia of inflammation or anemia of chronic diseaese  
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show Low serum iron, normal or low transferrin, low transferrin saturation, and high serum ferritin  
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Effect of anemia of inflammation on hepcidin   show
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Pathogenesis of Anemia of Inflammation   show
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Pathogenesis of anemia of renal failure   show
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show Normocytic-normochromic  
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Pathogenesis of the hereditary form of sideroblastic anemia   show
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Microscopic characteristics of red blood cells in anemia of renal failure   show
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show The incorporation of iron into heme is defective. May be acquired or hereditary.  
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Treatment for hereditary sideroblastic anemia   show
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Morphologic characteristics of red blood cells in sideroblastic anemia   show
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Effects on transferrin in sideroblastic anemia   show
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show Serum LDH is increased indicative of ineffective erythropoiesis.  
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show Acquired sideroblastic anemia  
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show Acquired sideroblastic anemia  
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show Lead inhibits delta-ALA dehydratase, coproporhyrinogen oxidase, and ferrochelatase important for heme synthesis and incorporation of iron. Coproporphyrin and ALA spill into the urine.  
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Inheritance pattern of Hereditary Hemochromatosis   show
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Gender predilection of hereditary hemochromatosis   show
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show Mutation of HFE gene  
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What is HFE?   show
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show HFE gene mutation  
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show HJV gene that codes for hemojuvelin  
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show HAMP gene that codes for Hepcidin  
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What genetic mutation is associated with Hemochromatosis Type 3?   show
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What genetic mutation is associated with Hemochromatosis Type 4?   show
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What are the two major types of HFE mutations associated with Hemochromatosis Type 1?   show
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What are the recommendations for individuals homozygous for the Cys28Tyr mutation?   show
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A disorder associated with hemojuvelin mutation that causes iron accumulation at an early age (age 15-30yrs). Associated clinical findings include diabetes, impotence, amenorrhea, infertility, and cardiac arrhythmias and heart failure.   show
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show Neonatal hemochromatosis  
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show Aceruloplasminemia  
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show (1) Repeated blood transfusionn (2) Ingestion of excessive amounts of dietary or supplmentary iron. Individuals with certain anemias, thalassemia, and siderblastic anemia, or porphyria cutanea tarda are at risk for secondary iron overload.  
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