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Hematology Lecture 10
Hematology Lecture 10 Red Cell Metabolism II - Hemoglobin
79
Biology
Undergraduate 3
11/28/2014

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Term
Hemoglobin molecule consists of four globin protein chains containing a heme component nestled in a ______ crevice
Definition
hydrophobic
Term
Heme is a ______ ring with ______ iron in the center
Definition
tetrapyrrole
ferrous
Term
____% of hemoglobin synthesis occurs during the nucleated stages of RBC maturation
Definition
65
Term
35% of hemoglobin synthesis occurs during the _______ stage
Definition
reticulocyte
Term
Globin
Definition
A tetramer of two pairs of unlike globin polypeptide chains
4 globular subunits
Term
Heme
Definition
4 groups
Each group contains a protoporphyrin ring plus ferrous iron (Fe2+)
Term
Hemoglobin structure consists of ______ and ______
Definition
Globin
Heme
Term
Normal Hemoglobin Structure is Dependent upon (3)
Definition
Adequate iron delivery and supply
Adequate synthesis of protporphyrins
Adequate globin synthesis
Term
What is the precursor of heme?
Definition
Protporphyrins
Term
What is the function of hemoglobin?
Definition
oxygen and CO2 transport
Term
3 Diseases Representing Defective Hemoglobin
Definition
Thalassemia
Iron Deficiency Anemia
Sideroblastic Anemia
Term
Which one of the following is Defective of Globin Chains:
Thalassemia
Iron Deficiency Anemia
Sideroblastic Anemia
Definition
Thalassemia
Term
Which of the following lacks iron:
Iron deficiency anemia
Thalassemia
Sideroblastic anemia
Definition
Iron Deficiency Anemia
Term
Which of the following is a disease representing lack of heme?
Thalassemia
Iron deficiency anemia
Sideroblastic anemia
Definition
Sideroblastic anemia
Term
Which of the following is the Most Common Disease representing Defective Hemoglobin:
Thalassemia
Iron Deficiency Anemia
Sideroblastic anemia
Definition
Iron Deficiency Anemia
Term
_____ of body's iron is bound to heme
Definition
2/3
Term
___-___ mg needed daily for erythropoiesis
Definition
20
25
Term
The average adult has a total body iron content of approximately ____ mg
Definition
3500
Term
_____ of the total body iron content is present as hemoglobin iron
Definition
2/3
Term
1/2 of the total body iron content is present as ______ iron
Definition
tissue
Term
90% of tissue iron is ____ iron
--Ferritin, hemosiderin
Definition
Storage
Term
10% of tissue iron is _____-_____
--Myoglobin, cytochrome enzymes
Definition
non-available
Term
True or False:
Daily iron intake, absorption, and losses are usually very small
Definition
True
Term
Is Body Iron repeatedly recycled?
Definition
Yes
Term
A healthy adult has a blood volume of
Definition
4500-5000 mL
Term
Will lose ____-____ mL whole blood per day due to RBC senescence
Definition
37
42
Term
2 mL of blood contains ____ mg of Iron
Definition
1
Term
____ - ____ mg of iron is needed each day to replace the iron lost by senescent RBCs
Definition
18.5
21
Term
Nearly 100% of iron from RBC turnover is taken up by the _____ ______ _____ and reutilized
Definition
mononuclear phagocytic system
Term
The small amount of iron lost through cellular shedding and sweating is ____ mg/day
This is replaced by diet
Definition
1
Term
The average western diet contains approximately ___ mg of iron/___ calories
Definition
6
1000
Term
The typical adult consumers ______ calories/day
Definition
2500
Term
Of the 15 mg of iron that is ingested/day, only ____-____% is absorbed
Definition
5
10
Term
Although excess iron is in our diet, ___ ______ continues to be a significant cause of morbidity in North America and throughout the world
Definition
iron deficiency
Term
Do the following contain High or Moderate Levels of Iron?
Organ meats
Wheat germ
Brewer’s yeast
Legumes
Definition
High
Term
Do the following contain High or Moderate Levels of Iron?
Muscle meats, fish, fowl
Prunes
Some green vegetables
Cereals
Definition
Moderate
Term
Do Phosphates and Milk increase or decrease absorption of Iron?
Definition
Decrease
Term
Are Antacids phosphates?
Definition
Yes
Term
Do the following low molecular weight compounds increase or decrease absorption of Iron?
Fructose
Amino acids
Ascorbic acid (vitamin C)
Definition
Increase
Term
5 Factors that affect daily iron requirements are:
Definition
Growth spurts
Menstruation
Pregnancy
Lactation and breast feeding
Iron deficiency
Term
Who is/are most prone to Iron Deficiency Anemia
Definition
Women
Children
Term
Where is Iron absorbed at?
Definition
Duodenum of the jejunum
Term
What is the most common dietary form of Iron?
Definition
Ferric (Fe+3)
Term
Ferric is converted into the ferrous (Fe+2) by what?
Definition
Acid of the stomach
Term
True or False:
Ferrous iron enters the mucosal cells of the intestine where it is converted back to ferric iron
Definition
True
Term
Iron complexes with ______ to form transferrin
Definition
apoferritin
Term
Iron complexes with apoferritin to form ______
Definition
transferrin
Term
In addition to the non-heme iron (ferrous and ferric molecules) in the diet, heme iron is also present as _____ and ______
Definition
Myoglobin
Hemoglobin
Term
True or False:
Heme iron is more readily absorbed than non-heme iron
Definition
True
Term
Derived from myoglobin and hemoglobin in dietary meat -
Iron is Absorbed by the ____ _____ as intact heme molecules and then freed from ______ _____ and utilized
Definition
mucosal cells
porphyrin ring
Term
_____ is delivered to the RBC precursor membrane by the protein carrier _____
Definition
Ferric (Fe3+)
Transferrin
Term
Iron crosses the membrane and enters the cytoplasm where it goes to the mitochondria. Here it is changed to _____
Definition
Ferrous (Fe+2)
Term
Excess iron in the cytoplasm aggregates as
Definition
Ferritin
Term
Ferritin
Definition
Storage form of iron
Amount will depend on the amount of iron needed to make hemoglobin
Term
Heme is a porphyrin ring with a single _______ iron attached in a hydrophobic pocket
Definition
ferrous
Term
With Heme Production, we want to protect Iron from _______
Definition
Oxidizing
Term
Heme synthesis begins in the _______ with the formation of ________ synthesis
Definition
mitochondria
protoporphyrin
Term
Glycine ALA + Succinyl Coenzyme A --> _____-_____ ____
Definition
delta-aminolevulinic acid
Term
The enzymatic steps for heme synthesis are influenced by
Definition
Erythropoietin
Vitamin B6
Term
ALA -->
Definition
PBG
Term
ALA --> PBG -->
Definition
URO
Term
ALA --> PBG --> URO -->
Definition
Cop
Term
ALA --> PBG --> URO --> Cop -->
Definition
Pro + Fe
Term
ALA --> PBG --> URO --> Cop --> Pro + Fe =
Definition
Heme
Term
Each enzyme step yields unstable substances called
Definition
porphyrinogens
Term
The porphyrinogens are oxidized to a more stable substance called _______
Definition
porphyrins
Term
After conversion of ________ __, iron is incorporated to yield heme
Definition
protophorphyrin IX
Term
______ ____ + ________ ___ -------------> Protoporphyrin IX (Protoporphyrin Ring)
Definition
Glycine ALA
Succinyl CoA
Term
Protoporphyrin IX + ______ --> Heme
Definition
Ferrochelatase
Term
Any block in the enzymatic pathway will result in the excess formation of a particular _____
Definition
Porphyrin
Term
Metabolic disorders are referred to as
Definition
porphyrias
Term
The porphyrin will accumulate in the
Definition
bone marrow
brain
liver
Term
True or False:
Porphyrin accumulation is Very Toxic to the brain
Definition
True
Term
True or False:
Porphyrias may be inherited or acquired
Definition
True
Term
If protoporphyrin synthesis is impaired, iron accumulates in the ______ as ____ _____
Definition
cytoplasm
ferritin aggregates
Term
Sideroblasts
Definition
iron-laden, nucleated RBC
Term
Siderocytes
Definition
Iron-laden, non-nucleated RBC
Term
Ringed Sideroblast
Definition
mitochondria become encrusted with iron and will be visible around the nucleus of the RBC precursor
Term
To visualize intracellular iron, must use _____ _____ stain
Definition
Prussian Blue
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