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Lecture 9 Test

Enter the letter for the matching Answer
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1.
What is the measurement of all iron bound to tranferrin in the body?
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2.
What is HFE?
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3.
What are the recommendations for individuals homozygous for the Cys28Tyr mutation?
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4.
What type of anemia is associated with ringed sideroblasts in the presence of dysplasia of myeloid and megakaryocytic cell lines.
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5.
Describe the effects of IRP-1 (iron regulatory proteins) on transferrin receptor (TfR1) syntehsis in the setting of low cellular iron.
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6.
A disorder due to mutations in the ceruloplasmin gene resulting in low serum ceruloplasmin.
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7.
MCV and MCHC of anemia of renal failure
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8.
What genetic mutation is associated with Hemochromatosis Type 4?
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9.
Treatment for hereditary sideroblastic anemia
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10.
(T or F) Ferritin levels in circulation is generally proportional to the size of the body reticuloendothelial cell iron store.
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11.
Morphologic characteristics of red blood cells in sideroblastic anemia
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12.
A disorder characterized by rapid massive iron buildup in the liver, heart and pancreas of a neonate resulting in stillbirth or death wihtin a few days of birth.
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13.
Effect of anemia of inflammation on hepcidin
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14.
What type of anemia is characterized by microcytic hypochromic anemia?
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15.
How is dietary heme transported across an enterocyte?
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16.
Describe the effects of IRP-1 (iron regulatory proteins) on ferritin syntehsis in the setting of low cellular iron.
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17.
How is percent of iron saturation?
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18.
Is a disorder of iron metabolism in which iron becomes unavailable for use in heme synthesis, resulting in normochromic/normocytic anemia. This type of anemia is usually associated with an underlying disease process. The resolves when the disease resolves
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19.
What are the two major types of HFE mutations associated with Hemochromatosis Type 1?
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20.
Dietary forms of iron
A.
Hepcidin is increased
B.
When cellular Fe is low, [4Fe-4S] clusters fail to form and IRP-1 (high affinity) then binds to the 5'-IRE of ferritin mRNA blocking translation of ferritin mRNA and decreasing the synthesis of ferritin.
C.
Serum iron (SI)
D.
When cellular Fe is low, [4Fe-4S] clusters fail to form and IRP-1 (high affinity) then binds to the 3'-IRE in tranferrin receptor (TfR1) mRNA, which stabilizes the IRE and increases TfR1 translation.
E.
(1) Ringed sideroblasts (2) Microcytic/hypochromic RBCs (3) Basophilic stippling
F.
Neonatal hemochromatosis
G.
Pyridoxine (Vitamin B6) supplement
H.
Acquired sideroblastic anemia
I.
Normocytic-normochromic
J.
(1) Cys28Tyr (substitution of tyrosine for cysteine - 85-90%) (2) His63Asp (substitution of aspartic acid for histidine - approximately 5%)
K.
Iron deficiency anemia
L.
SLC40A1 gene that codes for Ferroportin
M.
True.
N.
HFE forms a high-affinity complex with the transferrin receptor expressed on the surface of duodenal crypt cells, macrophages, and hepatocytes facilitating ransport of transferrin bound iron.
O.
Serum iron (SI) divided by the Total iron binding capacity (TIBC)
P.
Anemia of inflammation or anemia of chronic diseaese
Q.
Heme carrier protein 1 (HCP1)
R.
(1) Inorganic ferric iron (2) Heme iron
S.
Aceruloplasminemia
T.
(1) Regular screening for iron overload (2) Hemochromatosis DNA testing of relatives
Type the Answer that corresponds to the displayed Question.
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21.
Iron transport molecule that mobilizes iron from macrophages to be incorporated into transferrin
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22.
How is iron separated from dietary heme after absorption?
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23.
Where is hepcidin manufactred?
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24.
Ferritin not combined with iron
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25.
Normal range of percent iron saturation?
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26.
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.
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27.
Enzyme found in macrophages that catabolize heme and release ferrous iron from phagocytized erythrocytes
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28.
Gene mutation associated with Hemochromatosis Type 1
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29.
Anemia secondary to insufficient dietary iron or from blood loss.
Type the Question that corresponds to the displayed Answer.
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30.
A iron transport molecule that binds 2 Fe atoms.

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