Porphyrins And Hemoglobin

Front 1

Porphyrias are disorders associated with _______________.

Back 1

heme synthesis

Front 2

Hemoglobinopathies are disorders associated with ________________________.

Back 2

qualitative defects in the Hgb molecule

Front 3

Thalassemias are disorders of quantitative defects in the production of __________________.

Back 3

normal Hgb molecules

Front 4

Oxidation of UPG, CPG and protoporhyrinogen in heme synthesis produces what?

Back 4

porphyrins

Front 5

Precursors of porphyrins are excreted in the ________________.

Back 5

urine

Front 6

Uroporphyrinogen (UPG) is excreted in the _________.

Back 6

urine

Front 7

Coproporphyrinogen (CPG) is less soluble so it is excreted in the ________.

Back 7

urine and feces

Front 8

Protoporphyrin is the least water-soluble so it is excreted only in the ___________.

Back 8

feces

Front 9

The most common porphyria is....

Back 9

porphyria cutanea tarda

Front 10

Blistering skin lesions and excessive hair growth on the face are characteristics of what condition?

Back 10

porphyria cutanea tarda

Front 11

Each hgb molecule can carry up to _____ oxygen molecules.

Back 11

4

Front 12

Which iron is the iron binding component?

Back 12

Fe2+ (ferrous)

Front 13

The Bohr effect is when heme iron's affinity for oxyge is _________ when there is a decrease in pH.

Back 13

decreased

Front 14

Which Hgb is the main hgb presented in adults?

Back 14

Hgb A1

Front 15

Which Hgb is the main hgb presented in fetal life and the first few months of life?

Back 15

Hgb F

Front 16

This hemoglobin contains 2 alpha and 2 beta chains.

Back 16

Hgb A1

Front 17

This hemoglobin contains 2 alpha and 2 delta chains.

Back 17

Hgb A2

Front 18

This hemoglobin contains 2 alpha chains and 2 gamma chains.

Back 18

Hgb F

Front 19

Protoporphyrin is made in the ___________.

Back 19

mitochrondria

Front 20

Most hemoglobinopathies affect which chain in the Hgb molecule?

Back 20

beta

Front 21

Sickle cell disease is the presence of which Hgb?

Back 21

Hgb S

Front 22

Sickle cell trait gives resistance to what?

Back 22

P. falciparum

Front 23

In low oxygen, Hgb S looks like __________________.

Back 23

crescent shapes ("boat shaped")

Front 24

On electrophoresis, Hgb S migrates between Hgb ____ and Hgb _____.

Back 24

A and A2

Front 25

Which type of agar can you use for electrophoresis that would separate Hgb S, D and G?

Back 25

citrate agar at acidic pH

Front 26

Target cells and long hexagonal crystalline structures are seen in RBCs with what type of Hgb?

Back 26

Hgb C

Front 27

Deletion of 4 alpha genes results in what condition?

Back 27

Hgb Bart's hydrops fetalis

Front 28

Deletion of 3 alpha genes result in what?

Back 28

Hgb H disease

Front 29

Anisocytosis, hypochromia, basophilic stippling, target cells, and nucleated RBCs are seen in what type of thalassemia?

Back 29

B thal major

Front 30

Damage to muscle results in elevated plasma _____________ that is rapidly cleared by the kidneys which can cause acute renal failure because it is toxic to the renal tubules.

Back 30

myoglobin