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CPR > Heme Biochemistry (Part 2) > Flashcards

Heme Biochemistry (Part 2) Flashcards

1
Q

Heme has a ____ ring with iron in the center

A

Porphyrin

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2
Q

Porphyrin structure

A

4, 5-membered rings

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3
Q

Iron is in what form in Heme?

A

Ferrous (Fe2+)

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4
Q

Where does Heme biosynthesis occur?

A

Liver and erythroid cells of bone marrow

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5
Q

Where do the 3 phases of Heme Biosynthesis occur?

A
  1. Mitochondria
  2. Cytosol
  3. Mitochondria
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6
Q

What are the starting products for Phase 1 of heme synthesis?

A

Glycine + Succinyl CoA

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7
Q

During Phase 1 of heme synthesis, what enzyme acts on Glycine + Succinyl CoA?

A

delta - Aminolevulinic Acid (ALA) Synthase

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8
Q

What is the end product of Phase 1 of heme synthesis?

A

delta - Aminolevulinic Acid (ALA)

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9
Q

What are the starting products for Phase 2 of heme synthesis?

A

ALA + ALA

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10
Q

For Phase 2 of heme synthesis, what enzyme combines ALA + ALA?

A

ALA Dehydratase

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11
Q

During Phase 2 of heme synthesis, ALA + ALA using ALA Dehydratase creates?

A

Porphobilinogen

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12
Q

During Phase 2 of heme synthesis, what is the end product?

A

Coproporphyrinogen III

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13
Q

During Phase 3 of heme synthesis, protoporphyrin is converted to?

A

Heme

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14
Q

What enzyme during Phase 3 of heme synthesis converts protoporphyrin to Heme?

A

Ferrochelatase

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15
Q

What does Ferrochelatase enzyme add to protoporphyrin?

A

Ferrous (Fe2+)

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16
Q

What does delta - aminolevulinic acid synthase require?

A

Vitamin B6

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17
Q

Where does Heme have negative feedback on its synthesis?

A

Delta - Aminolevulinic Acid Synthase

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18
Q

Lead poisoning inactivates which 2 enzymes in Heme synthesis?

A

ALA Dehydratase

Ferrochelatase

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19
Q

With lead poisoning, what substrates accumulate?

A

ALA Dehydratase – ALA accumulates

Ferrochelatase – Protoporphyrin IX

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20
Q

What does lead poisoning cause?

A

Anemia and impacts ATP synthesis

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21
Q

ALA accumulation is ____

A

Neurotoxic

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22
Q

Porphyrias

A

Defects in heme synthesis

23
Q

What enzyme is deficient with Acute Intermittent Porphyria?

A

Porphobilinogen Deaminase

24
Q

Acute Intermittent Porphyria symptoms

A

Hepatic - neurologic

25
Q

What enzyme is deficient with Congenital Erythropoietic Porphyria?

A

Uroporphyrinogen III Cosynthase

26
Q

Congenital Erythropoietic Porphyria symptoms

A

Erythropoietic - produces a red color of urine and teeth and skin photosensitivity

27
Q

What enzyme is deficient with Porphyria Cutanea Tarda?

A

Uroporphyrinogen Decarboxylase

28
Q

Porphyria Cutanea Tarda symptoms

A

Hepatoerythropoietic - neurologic and skin sensitivities

29
Q

What enzyme is deficient with Variegate Porphyria?

A

Protoporphyrinogen Oxidase

30
Q

Variegate Porphyria symptoms

A

Hepatic - neurologic

31
Q

Heme is degraded to Biliverdin using what enzyme?

A

Heme oxygenase

32
Q

Heme oxygenase takes Heme to Biliverdin using?

A

Oxygen

33
Q

Heme oxygenase releases ____ & ____from heme

A

Iron (ferrous)

CO2

34
Q

Biliverdin is converted to Bilirubin using what enzyme?

A

Biliverdin Reductase

35
Q

Biliverdin Reductase requires _____ to convert Biliverdin to Bilirubin

A

NADPH

36
Q

Bilirubin is sent into the blood stream and is bound to?

A

Albumin

37
Q

In the liver, Bilirubin is combined with?

A

UDP - Glucuronate

38
Q

Bilirubin is combined with UDP-Glucuronate using what enzyme?

A

UDP - GlucuronylTransferase

39
Q

What is the rate limiting step of Heme degradation?

A

UDP - GlucuronylTransferase combining UDP-Glucuronate and Bilirubin to form Bilirubin Diglucuronide

40
Q

Bilirubin + UDP - Glucurornate =

A

Bilirubin Diglucuronide

41
Q

Bilirubin Diglucuronide is then sent to?

A

Gallbladder

42
Q

Once Bilirubin Diglucuronide is in the intestines, it is converted to?

A

Urobilinogen

43
Q

If urobilinogen is sent to the kidneys, it becomes?

A

Urobilin

44
Q

Urobilin causes?

A

Urine to be yellow

45
Q

If urobilinogen remains in the intestines, it becomes?

A

Stercobilin

46
Q

Stercobilin causes?

A

Red, brown color to feces

47
Q

Jaundice means

A

Hyperbiliurbinemia

48
Q

What are the 3 locations of disorders for jaundice?

A

Pre-hepatic
Intra-hepatic
Post-hepatic

49
Q

Pre-hepatic jaundice is increased?

A

UNconjugated Bilirubin

50
Q

What are the 2 common diseases with intra-hepatic jaundice disorders?

A

Criggler-najjar syndrome

Gilbert syndrome

51
Q

What enzyme is usually absent or deficient with intra-hepatic disorders?

A

UDP - GlucuronylTransferase

52
Q

Post-hepatic jaundice is increased?

A

CONJUGATED Bilirubin

53
Q

What symptoms will post-hepatic jaundice patients have?

A

DARK urine and pale stool

54
Q

Hepatitis: increased unconjugated or conjugated bilirubin in the blood?

A

BOTH

CPR (48 decks)

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