globin synthesis Flashcards

1
Q

ref range of hgb for women (conventional)

A

12-15 g/dL

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1
Q

give the symptoms present in congenital erythropoietic porphyria

A
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
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3
Q

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

A

chromosome 11

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4
Q

3 embryonic hemoglobin

A

gower I
gower II
portland

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5
Q

gower II contains

A

alpha

epsilon

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6
Q

production of globin chains takes place in

A

pronormoblast-reticulocyte

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7
Q

globin synthesis is controlled by

A

genetic control

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8
Q

hbA is composed of?

lifespan?

A

2 alpha
2 beta

6 mos-adulthood

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8
Q

other name for methemoglobin reductase

A

cytochrome b5 reductase

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9
Q

hbF is not found in adults but in?

A

F cells

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10
Q

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

A

hbA1

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11
Q

portland contains

A

zeta

gamma

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12
Q

hb ref range for men (SI)

A

2.17-2.79 mmol/L

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13
Q

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

A

gower I
gower II
portland

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15
Q

hgb which is predominant in normal adult

A

hbA

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16
Q

ref range of hgb for newborns (conventional)

A

16.5-21.5 g/dL

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17
Q

hgb which is predominant at birth

A

hbF

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18
Q

inherited/acquired disorders of heme synthesis

A

porphyrias

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19
Q

chromosome which dictates alpha and zeta

A

chromosome 16

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21
Q

ref range of hgb for newborns (SI)

A

2.17-4.03 mmol/L

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24
Q

oxyhgb is seen in?

what color of blood?

A

arterial blood

bright red

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25
Q

methemoglobin is increased in (3)

A

presence of oxidants
decreased methemoglobin reductase
hbM disease

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26
Q

ref range of hgb for women (SI)

A

1.86-2.48 mmol/L

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27
Q

deoxyhgb is seen in?

what color?

A

venous blood

dark red

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28
Q

color of blood with carboxyhgb

A

cherry red

29
Q

rbcs w/ Shb have (2)

A

normal survival

osmotic fragility

30
Q

6 oxidizing agents

A
aniline derivatives
acetanilide
acetaphenetidin
chlorates
k ferricyanide
k permanganate
31
Q

mRNA which is translated more efficiently

A

beta-globin mRNA

33
Q

this results when carboxyhgb is not capable of transporting o2

A

hypoxia

34
Q

most common neurologic porphyria

A

acute intermittent porphyria

36
Q

methemoglobin contains what form of iron

A

ferric

37
Q

hb + ? = methemoglobin

A

oxidizing agents

38
Q

deficiency caused by the defect in variegate porphyrias

A

ferrochelatese deficiency

39
Q

methemoglobin is reversible, T/F

A

T

40
Q

porphyria which appears in adults due to excessive alcohol intake

A

porphyria cutanea tards

41
Q

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

A

ascorbic acid

methylene blue

43
Q

treatment for methemoglobin

A

removal of offending substrate

administration of ascorbic acid and methylene blue

44
Q

formed by irreversible oxidation of hgb by certain drugs and chemicals

A

sulfhemoglobin

45
Q

hgb which contains 2 alpha and 2 gamma

A

hbF

46
Q

hba2 is composed of

A

2 alpha

2 beta

47
Q

4 drugs/chemicals which results to sulfhemoglobin

A

sulfinamides
acetanilide
phenazopyridine
phenacetin

48
Q

primary cause of porphyrias

A

specific enzyme deficiency

49
Q

gower I contains

A

zeta

epsilon

50
Q

porphyrias can be extreted through (3)

A

urine
stool
deposited in tissue

51
Q

3 symptoms of neurologic porphyrias

A

abdominal pain
psychotic behavior
neuromuscular difficulties

52
Q

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

A

F, unmodified/ normal hgb

53
Q

reaction with sulfhgb is reversible, T/F

A

F, irreversible

54
Q

enzyme defect in variegate porphyria

A

heme synthetase

55
Q

substances increased in variegate porphyrias

A

iron

protorporphyrin IX

56
Q

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

A

lead intoxiation

57
Q

enzymes absent in lead intoxication

A

ferrochelatase

coproporphyrinogen oxidase

58
Q

other name of d-ALA dehydrogenase

A

PBG synthetase

60
Q

term used if the teeth is fluorescent

A

erythrodontia

61
Q

most common cutaneous porphyria

A

porphyria cutanea tard

62
Q

most severely affected enzyme in lead intoxication

A

d-ALA dehydrogenase

65
Q

blue discoloartion if skin

A

cyanosis

74
Q

hb ref range for men (conventional)

A

14-18 g/dL

77
Q

methemoglobin gives what color of blood

A

chocolate brown color

79
Q

methemoglobin is also known as

A

ferrihgb

87
Q

what are the 3 abnormal hgbs

A

carboxyhgb
methgb
sulfhgb

90
Q

carboxyhgb is reversible, T/F

A

F, irreversible