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HEME/LYMPH > Heme, Iron and Bilirubin Metabolism > Flashcards

Heme, Iron and Bilirubin Metabolism Flashcards

1
Q

hemoglobin

A

heme - iron

globin - two pairs of chains

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2
Q

heme

A

porphrin ring with one iron chelated by 4 nitrogens

reversible oxygen attachment

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3
Q

heme synthesis

A

succinyl CoA and glycine > ALA
ENZ - ALA synthase
-rate limiting step

heme - negative feedback inhibitor

2x ALA > prophobilinogen
ENZ - PBG synthase - ALA dehydratase

condensation of 4 x porphobilinogen > protoporphyrin

Fe added to protoporphyrin
ENZ - ferrochelatase

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4
Q

sideroblastic anemia

A

mutation of ALA synthase

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5
Q

porphyria

A

mutation in porphobilinogen synthase or PBG deaminase

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6
Q

Pb poisoning

A

affects ALA dehydratase

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7
Q

ALA synthase

A

rxn in mitochondria

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8
Q

ALA dehydratase

A

rxn in cytosol

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9
Q

ferrochelatase

A

rxn back into mitochondria

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10
Q

alpha globins

A

chromosome 16

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11
Q

beta globins

A

chromosome 11

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12
Q

thalassemia

A

disruption of balance of globin chain synthesis

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13
Q

Hb gower

A

embryonic

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14
Q

portland Hb

A

fetal

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15
Q

HbA

A

in adults

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16
Q

majority HbA

A

alpha2beta2

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17
Q

heme and globin

A

combine in cytoplasm

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18
Q

transferrin

A

carries iron

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19
Q

ferrous

A

Fe2+

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20
Q

Hg only binds

A

ferrous - Fe2+

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21
Q

ferrous bonds

A

4 - heme
1 - globin
1 - oxygen

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22
Q

iron absorption

A

duodenum

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23
Q

ferric

A

Fe3+

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24
Q

ferric reductase

A

DcytB

-Fe3+ to Fe2+

25
Q

DMT1

A

cotransport Fe2+/H+ into cell

26
Q

ferroportin

A

transport Fe2+ out of cell

regulated by hepcidin levels

27
Q

hepicidin

A

regulator of ferroportin

-Fe transport out of cell

28
Q

hemochromatosis

A

high levels of Fe

29
Q

heme oxygenase

A

inside cells

-splits heme iron and releases free Fe3+

30
Q

high Fe levels

A

high hepcidin

-decreased ferroportin

31
Q

low Fe levels

A

low hepcidin

  • increased ferroportin
  • more absorption from gut
32
Q

transport in blood

A

Fe3+ on transferrin

33
Q

ferritin

A

storage of Fe

Fe + apoferritin

34
Q

apoferritin

A

iron buffer system

35
Q

HAMP mutation

A

decreased hepcidin

iron overload and hemochromatosis

36
Q

RBC breakdown

A

120 days
-in spleen

no spleen - to liver

37
Q

RBC metabolism

A

glycolysis - ATP and NADH

pentose phosphate - NADPH to maintain reduced glutathione

38
Q

fava beans

A

RBC breakdown

39
Q

polycythemia

A

high RBCs

40
Q

anemia

A

low RBC

41
Q

hemoglobin in kidney

A

nephrotoxic
-damages renal tubule cells

Tx dialysis

42
Q

red pulp

A

of spleen
-RBC graveyard

as age - get more rigid

heme > bilirubin
Fe recycled

43
Q

Hg breakdown

A

macrophage
-porphyrin ring broken down by heme oxidase

give biliverdin and CO

44
Q

methemoglobin

A

Hg carrying ferric iron - Fe3+

loses ability to carry oxygen

methemoglobin reductase - solves problem - metHg back to normal Hg

45
Q

caboxyhemoglobin

A

Hg bound to CO

46
Q

phenacetin

A

or sulfonamides

leads to sulfhemoglobin

47
Q

HgA2

A

2 alpha and 2 delta

48
Q

HgF

A

2 alpha and 2 gamma

fetal

49
Q

sickle cell trait

A

heterozygote

HgS

50
Q

sickle cell disease

A

homozygote

HgS

51
Q

point mutation in globin chain

A

sickle cell disease

52
Q

inherited defect in regulation of globin chain synthesis

A

thalassemia

-underproduced globin production

53
Q

microcytic hypochromic anemia

A

thalassemia

has prominent target cells on peripheral smear

decreased MCV and MCH

54
Q

Hg H disease

A

loss of 3 of 4 alpha globin genes

55
Q

alpha-thalassemia major

A

loss of all 4 alpha globin genes

hydrops fetalis
Hg barts forms

56
Q

cooleys anemia

A

beta thalassemia major

57
Q

beta thalassemia

A

protection against malaria

58
Q

hair on end skull radiograph

A

beta thalassemia

HEME/LYMPH (11 decks)

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