Heme Metabolism Flashcards

1
Q

What are the two starting materials of heme metabolism

A

Glycine and succinyl CoA

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2
Q

Where does heme metabolism start

A

Mitochondria

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3
Q

What is the limiting reaction catalysed by

A

¿-ALA synthase

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4
Q

What serves as a coenzyme for ALA synthase

A

Pyridoxine phosphate(PLP)

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5
Q

How many molecules of ALA condense in the cytosol

A

2

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6
Q

Which enzyme is zinc containing and is invited by lead, particularly.

A

ALA Dehydratase

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7
Q

What is the first pathway intermediate that has a pyrrole ring

A

Porphobilinogen

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8
Q

What does the condensation of four porphobilinigen(PBG) form? And what enzyme catalyses it

A

Hydroxymethylbilane
Catalysed by: porphobilinogen deaminase

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9
Q

What’s is protoporphrinogen IX oxidised to

A

Porphyrinogen IX

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10
Q

What catalyses the final step in hen synthesis

A

Ferrochelatase

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11
Q

Which step involves the incorporation of ferrous iron

A

Step from porphyrinigen to heme

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12
Q

What acts both as a repressor and inhibitor of ALA synthase

A

Heme
Hematin

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13
Q

Which two enzymes are einhiyed by lead

A

ALA dehydratase
Ferrochelatase

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14
Q

What does hemin inhibit

A

ALA Synthase

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15
Q

What are epossible triggers of porphyrias

A

Alcohol
Smoking
Lead poisoning or iron
Hiv
Eating disorders, stress, sunlight

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16
Q

What are epossible triggers of porphyrias

A

Alcohol
Smoking
Lead poisoning or iron
Hiv
Eating disorders, stress, sunlight

17
Q

Mention and define two major types of porphyria

A

Cutaneous porphyria….skin, oversensitive to sunlight, shrunken gums
Acute porphyria….affecting nervous system

18
Q

Write down the heme metabolism structure and diseases associated either it’s enzymes

A
19
Q

Where does Hb degrade

A

In ER og reticuloendothelial cells of liver, spleen, bone marrow and skin

20
Q

What dies Hb degrade into

A

Globins
Heme-bilirubin
Fe+2