Heme Biochemistry (Part 2) Flashcards

(54 cards)

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
What enzyme is deficient with Congenital Erythropoietic Porphyria?
Uroporphyrinogen III Cosynthase
26
Congenital Erythropoietic Porphyria symptoms
Erythropoietic - produces a red color of urine and teeth and skin photosensitivity
27
What enzyme is deficient with Porphyria Cutanea Tarda?
Uroporphyrinogen Decarboxylase
28
Porphyria Cutanea Tarda symptoms
Hepatoerythropoietic - neurologic and skin sensitivities
29
What enzyme is deficient with Variegate Porphyria?
Protoporphyrinogen Oxidase
30
Variegate Porphyria symptoms
Hepatic - neurologic
31
Heme is degraded to Biliverdin using what enzyme?
Heme oxygenase
32
Heme oxygenase takes Heme to Biliverdin using?
Oxygen
33
Heme oxygenase releases ____ & ____from heme
Iron (ferrous) | CO2
34
Biliverdin is converted to Bilirubin using what enzyme?
Biliverdin Reductase
35
Biliverdin Reductase requires _____ to convert Biliverdin to Bilirubin
NADPH
36
Bilirubin is sent into the blood stream and is bound to?
Albumin
37
In the liver, Bilirubin is combined with?
UDP - Glucuronate
38
Bilirubin is combined with UDP-Glucuronate using what enzyme?
UDP - GlucuronylTransferase
39
What is the rate limiting step of Heme degradation?
UDP - GlucuronylTransferase combining UDP-Glucuronate and Bilirubin to form Bilirubin Diglucuronide
40
Bilirubin + UDP - Glucurornate =
Bilirubin Diglucuronide
41
Bilirubin Diglucuronide is then sent to?
Gallbladder
42
Once Bilirubin Diglucuronide is in the intestines, it is converted to?
Urobilinogen
43
If urobilinogen is sent to the kidneys, it becomes?
Urobilin
44
Urobilin causes?
Urine to be yellow
45
If urobilinogen remains in the intestines, it becomes?
Stercobilin
46
Stercobilin causes?
Red, brown color to feces
47
Jaundice means
Hyperbiliurbinemia
48
What are the 3 locations of disorders for jaundice?
Pre-hepatic Intra-hepatic Post-hepatic
49
Pre-hepatic jaundice is increased?
UNconjugated Bilirubin
50
What are the 2 common diseases with intra-hepatic jaundice disorders?
Criggler-najjar syndrome | Gilbert syndrome
51
What enzyme is usually absent or deficient with intra-hepatic disorders?
UDP - GlucuronylTransferase
52
Post-hepatic jaundice is increased?
CONJUGATED Bilirubin
53
What symptoms will post-hepatic jaundice patients have?
DARK urine and pale stool
54
Hepatitis: increased unconjugated or conjugated bilirubin in the blood?
BOTH