heme metabolism Flashcards

1
Q

heme structure

A

porphyrin ring + iron + protoporphyrin 9

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2
Q

what is heme

A

an iron-containing cofactor for hemoglobin

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3
Q

what is the committed step of heme synthesis

A

glycine + succinyl-CoA
–(ALAS)–>
ALA
->
->
->
heme

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4
Q

what catalyzes the committed step of heme synthesis, & how does this differ by location

A

ALAS1 in liver
ALAS2 in bone marrow

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5
Q

heme synthesis pathway

A
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6
Q

what is HIF

A

Hypoxia-Inducible (transcription) Factor

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7
Q

HIF in normoxia pathway

A

HIF-OH
->
ubiquinated by VHL1
->
degradation by protease

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8
Q

HIF in hypoxia pathway

A

HIF increases transcription of gene Epo
->
Epo binds EpoR in bone marrow
->
increases RBC production

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9
Q

Epo is a _____ that controls _____

A

cytokine
erythropoiesis

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10
Q

what is the Bohr effect

A

^ [H+], CO2 = ^ O2 release from hemoglobin

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11
Q

what is the “T state” of hemoglobin

A

low O2 affinity

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12
Q

what does 2,3BPG binding to hemoglobin do

A

^ O2 release from hemoglobin

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13
Q

high altitude hyperventilation (altitude sickness) causes

A

respiratory alkalosis

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14
Q

treatment for altitude sickness

A

carbonic anhydrase inhibitor (blocks CO2 formation)

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15
Q

treatment for CO poisoning, & why

A

administer high O2

to outcompete CO that was taking O2 binding site on hemoglobin

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16
Q

porphyria

-inheritance
-caused by
-hallmark

A

autosomal dominant

mutation in heme biosynthesis

purple urine

17
Q

_____ limits rate of heme formation in erythroid cells

18
Q

4 types of hemoglobinopathies

A

alpha thalassemias (anemia)
beta thalassemias (anemia)
sickle cell anemia
HbC disease

19
Q

sickle cell disease

-inheritance
-caused by

A

homozygous recessive

mutation in HBB gene

20
Q

heme catabolism pathway in macrophage

21
Q

heme catabolism pathway in liver

22
Q

_____ can result in jaundice

A

hyperbilirubinemia

23
Q

3 common types of jaundice

A

hemolytic
hepatocellular
obstructive/cholestatic

24
Q

Gilbert’s syndrome

-what is it
-genetic cause

A

isolated hyperbilirubinemia (unconjugated)

mutations of UGT1A1 gene

25
Q

how does phototherapy help jaundiced newborns

A

photoisomerizes H-bond of heme
->
increases water solubility of unconjugated / indirect bilirubin = more excretion