Lecture 38 Flashcards

(21 cards)

1
Q

What is present to shuttle Iron into the cell? What oxidation state can it be in? Is it complete absorption?

A
  • active transport system
  • +2 or +3
  • no - iron absoprtion isn’t very efficient
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2
Q

What are some iron transporters and how much iron do they carry?

A

Ferritin (4500 Fe), Transferrin (2 Fe), Lactoferrin (to get iron into milk)

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3
Q

How much iron is in a 70 kg human? Where is it mainly?

A
  • 3-4 g

- hemoglobin or stored ferritin in liver, reticuloendothelial cells and muscle

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4
Q

What convert Fe+2 to Fe+3? Why is this important?

A
  • ferroxidases

- in order for RBCs to carry O2, Fe must be +2

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5
Q

What is iron deficiency anemia?

A
  • Poorly developed RBCs when FE not available for Hgb synthesis
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6
Q

What is Hemochromatosis?

A
  • Excessive Fe absorption and storage leads to liver and other tissue damage
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7
Q

What is a heme? Where does synthesis occur?

A
  • A porphyrin ring with Fe

- Synthesis occurs in all tissues, enriched in marrow and liver

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8
Q

Heme synthesis begins and ends in what compartment? Where do the intermediate steps occur?

A
  • mitochondria

- cytosol

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9
Q

What two compounds combine in the first step of Heme synthesis? What is the enzyme? What inhibits it? What is the cofactor?

A
  • succinyl CoA and glycine
  • ALA synthase
  • Heme
  • Pyridoxal phosphate (a b vitamin)
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10
Q

What is the general name for the intermediates occurring in the cytosol during heme synthesis?

A
  • porphorinogen
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11
Q

How does the ALA synthase get into the mitochondria? What inhibits transcription of this enzyme?

A
  • a basic signalling sequence is present at its end, a chaperone protein and ATP attach and it gets shuttled in
  • heme
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12
Q

What happens when 3 H2 are removed from Protoporphyrinogen IX to make Protoporphyrin IX (porphorinogens)?

A
  • more double bonds - double bond structure changes
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13
Q

What is a derangement in a number of genes or poryphyrin synthesis called that occur in a number of species called? Dominant or recessive? What happens in case of dominant? What can happen? What do they primarily affect? Where does most heme synthesis occur?

A
  • porphryias
  • can be both
  • gain of function and overproduction of intermediates
  • Defective Hgb and other heme-protein synthesis
  • buildup of precursors to blockage point (reddish, brownish, light sensitive)
  • nervous system, skin, liver, blood
  • liver
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14
Q

What does auto-oxidation of Fe2+ in hemoglobin to Fe3+ produce? What color is triple state? What enzyme returns to 2+ state? What is needed for this reaction?

A
  • inactive methemoglobin and superoxide
  • brown
  • methemoglobin reductase (MR)
  • NADH
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15
Q

Where is hemoglobin catabolized? What happens to the protein (globin)? What happens to the AA? What happens to Heme?

A
  • reticuloendothelial (RE) cells of spleen and bone marrow
  • cleaved off, hydrolyzed, exported as AA
  • reused by organism
  • degraded and must be excreted (3-5 mg/kg/day)
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16
Q

What breaks open Heme ring structure and removes Fe3+? What does it use? What is made? What converts Biliverdin to bilirubin? what does it use? What color is biliverdin? Bilirubin?

A
  • heme oxygenase
  • NADPH
  • Biliverdin IXa
  • biliverdin reductase
  • NADPH
  • blue-green
  • yellow
17
Q

Bilirubin still isn’t that soluble - how does body make it so? What enzyme does this? Where primarily? Why is this good?

A
  • glucuronate conjugation
  • Bilirubin UDP glucuronyltransferase
  • liver
  • bilirubin can be excreted via the bile ducts > gi tract > fisces
18
Q

Where does liver take up bilirubin from? Where does it move it? What does it do with it there?

A
  • blood
  • smooth ER
  • conjugates it, excretes into bile
19
Q

Most of bilirubin is excreted as what? What percentage ends up in periperal blood? What percentage is sent back to liver?

A
  • urobilinogen
  • 15%
  • 14%
20
Q

Can disorders be measured using conjugated and unconjugated bilirubin in blood? What does elevated conjugated bilirubin mean? Elevated unconjugated?

A
  • yes
  • problem in excretion
  • problem in transport or conjugation in the liver
21
Q
  • What enzyme carries out the second reaction of heme synthesis? where? what inhibits it?
A
  • ALA dehydratase
  • cytoplasm
  • lead