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Lecture 9 Quiz

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1. What genetic mutation is associated with Hemochromatosis Type 2A?




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2. Pathogenesis of lead poisoning induced sideroblastic anemia




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3. What type of anemia is the most common and often the earlierst evidence of myelodysplastic syndrome?




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4. Portion of the intestine that absorps iron




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5. What type of anemia is associated with ringed sideroblasts in the presence of dysplasia of myeloid and megakaryocytic cell lines.




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6. How is percent of iron saturation?




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7. Pathogenesis of Anemia of Inflammation




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8. How is non-heme iron or ferric iron transported across an enterocyte?




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9. What are iron regulatory proteins (IRP-1 and IRP-2)?




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10. Gene mutation associated with Hemochromatosis Type 1




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11. Heme carrier protein 1 (HCP1)




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12. True.




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13. Juvenile hemochromatosis




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14. HAMP gene that codes for Hepcidin




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15. Low serum iron, normal or low transferrin, low transferrin saturation, and high serum ferritin




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16. 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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17. IRP-1 switches between two forms: high and low affinity forms. IRP-2 functions like IRP-1, but is either produced or not produced. There are no high or low affinity forms of IRP-2.




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18. Heme oxygenase 1




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19. (1) Regular screening for iron overload (2) Hemochromatosis DNA testing of relatives




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20. X-linked trait with variable expression resulting in decreased activity of ALA synthesis.





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