globin synthesis

Question 1

ref range of hgb for women (conventional)

Answer 1

12-15 g/dL

Question 1

give the symptoms present in congenital erythropoietic porphyria

Answer 1

deformity of fingers/nails
red discoloration of teeth
associted with severe photosensitivity
scarring
chronic hemolysis
excessive hair growth
teeth and urine are fluorescent under uv

Question 3

chromosome which dictates other proteins(beta, gamma, delta, epsilon)

Answer 3

chromosome 11

Question 4

3 embryonic hemoglobin

Answer 4

gower I
gower II
portland

Question 5

gower II contains

Answer 5

alpha

epsilon

Question 6

production of globin chains takes place in

Answer 6

pronormoblast-reticulocyte

Question 7

globin synthesis is controlled by

Answer 7

genetic control

Question 8

hbA is composed of?

lifespan?

Answer 8

2 alpha
2 beta

6 mos-adulthood

Question 8

other name for methemoglobin reductase

Answer 8

cytochrome b5 reductase

Question 9

hbF is not found in adults but in?

Answer 9

F cells

Question 10

formed by non enzymatic rxnof sugrs to amino grps of globin chain

Answer 10

hbA1

Question 11

portland contains

Answer 11

zeta

gamma

Question 12

hb ref range for men (SI)

Answer 12

2.17-2.79 mmol/L

Question 13

what are the hgb present at 1st 3 mos of embryonic development

Answer 13

gower I
gower II
portland

Question 15

hgb which is predominant in normal adult

Answer 15

hbA

Question 16

ref range of hgb for newborns (conventional)

Answer 16

16.5-21.5 g/dL

Question 17

hgb which is predominant at birth

Answer 17

hbF

Question 18

inherited/acquired disorders of heme synthesis

Answer 18

porphyrias

Question 19

chromosome which dictates alpha and zeta

Answer 19

chromosome 16

Question 21

ref range of hgb for newborns (SI)

Answer 21

2.17-4.03 mmol/L

Question 24

oxyhgb is seen in?

what color of blood?

Answer 24

arterial blood

bright red

Question 25

methemoglobin is increased in (3)

Answer 25

presence of oxidants
decreased methemoglobin reductase
hbM disease

Question 26

ref range of hgb for women (SI)

Answer 26

1.86-2.48 mmol/L

Question 27

deoxyhgb is seen in?

what color?

Answer 27

venous blood

dark red

Question 28

color of blood with carboxyhgb

Answer 28

cherry red

Question 29

rbcs w/ Shb have (2)

Answer 29

normal survival | osmotic fragility

Question 30

6 oxidizing agents

Answer 30

``` aniline derivatives acetanilide acetaphenetidin chlorates k ferricyanide k permanganate ```

Question 31

mRNA which is translated more efficiently

Answer 31

beta-globin mRNA

Question 33

this results when carboxyhgb is not capable of transporting o2

Answer 33

hypoxia

Question 34

most common neurologic porphyria

Answer 34

acute intermittent porphyria

Question 36

methemoglobin contains what form of iron

Answer 36

ferric

Question 37

hb + ? = methemoglobin

Answer 37

oxidizing agents

Question 38

deficiency caused by the defect in variegate porphyrias

Answer 38

ferrochelatese deficiency

Question 39

methemoglobin is reversible, T/F

Answer 39

T

Question 40

porphyria which appears in adults due to excessive alcohol intake

Answer 40

porphyria cutanea tards

Question 41

these 2 can reduce the iron (methemoglobin) in its reduced form

Answer 41

ascorbic acid | methylene blue

Question 43

treatment for methemoglobin

Answer 43

removal of offending substrate | administration of ascorbic acid and methylene blue

Question 44

formed by irreversible oxidation of hgb by certain drugs and chemicals

Answer 44

sulfhemoglobin

Question 45

hgb which contains 2 alpha and 2 gamma

Answer 45

hbF

Question 46

hba2 is composed of

Answer 46

2 alpha | 2 beta

Question 47

4 drugs/chemicals which results to sulfhemoglobin

Answer 47

sulfinamides acetanilide phenazopyridine phenacetin

Question 48

primary cause of porphyrias

Answer 48

specific enzyme deficiency

Question 49

gower I contains

Answer 49

zeta | epsilon

Question 50

porphyrias can be extreted through (3)

Answer 50

urine stool deposited in tissue

Question 51

3 symptoms of neurologic porphyrias

Answer 51

abdominal pain psychotic behavior neuromuscular difficulties

Question 52

sulfhemoglobin cannot be convrted to modified hgb; it persists for the life of the cell, T/F

Answer 52

F, unmodified/ normal hgb

Question 53

reaction with sulfhgb is reversible, T/F

Answer 53

F, irreversible

Question 54

enzyme defect in variegate porphyria

Answer 54

heme synthetase

Question 55

substances increased in variegate porphyrias

Answer 55

iron | protorporphyrin IX

Question 56

commonly acquired porphyria that results from block in several heme biosynthetic enzyme

Answer 56

lead intoxiation

Question 57

enzymes absent in lead intoxication

Answer 57

ferrochelatase | coproporphyrinogen oxidase

Question 58

other name of d-ALA dehydrogenase

Answer 58

PBG synthetase

Question 60

term used if the teeth is fluorescent

Answer 60

erythrodontia

Question 61

most common cutaneous porphyria

Answer 61

porphyria cutanea tard

Question 62

most severely affected enzyme in lead intoxication

Answer 62

d-ALA dehydrogenase

Question 65

blue discoloartion if skin

Answer 65

cyanosis

Question 74

hb ref range for men (conventional)

Answer 74

14-18 g/dL

Question 77

methemoglobin gives what color of blood

Answer 77

chocolate brown color

Question 79

methemoglobin is also known as

Answer 79

ferrihgb

Question 87

what are the 3 abnormal hgbs

Answer 87

carboxyhgb methgb sulfhgb

Question 90

carboxyhgb is reversible, T/F

Answer 90

F, irreversible