Heme Synthesis Flashcards

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1
Q

Name the heme proteins

A

hemoglobin, myoglobin, cytochrome, catalase, tryptophan pyrrolase

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2
Q

What are porphyrins

A

Intermediates during heme synthesis

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3
Q

What joins pyrrole rings together

A

methenyl

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4
Q

Which atom in the pyrrole ring attaches to metal ion

A

Nitrogen

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5
Q

Porphyrin Intermediates during heme synthesis

A

Uroporphyrin, Coporphyrin and Protoporphyrin

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6
Q

Side chains of uroporphyrin

A

4 acetyl and 4 proprionyl

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7
Q

Side chains of coporphyrin

A

4 methyl and 4 proprionyl

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8
Q

Side chains of protoporphyrin

A

4 methyl, 2 vinyl and 2 propionyl

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9
Q

Uroporphyrin is excreted in urine or feces?

A

Urine

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10
Q

Coporphyrin and Protoporphyrin is excreted in urine/feces?

A

Feces

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11
Q

Which type (I or III) of uroporphyrin is important in humans

A

Type III

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12
Q

Which type of cells cannot synthesize heme

A

Red blood cells

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13
Q

What is the rate limiting enzyme of heme synthesis in the liver

A

delta- aminolevulinic acid synthase

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14
Q

What is the enzyme delta- aminolevulinic acid synthase inhibited by

A

Heme

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15
Q

In heme synthesis, which reaction requires pyridoxal phosphate (vitamin B6)

A

Conversion of glycine and succinyl coA to delta- aminolevulinic acid synthase

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16
Q

Which two reactions in heme synthesis are inhibited by heavy metal poisoning (eg. lead)

A
  1. Conversion of delta- aminolevulinic acid synthase to porphobilinogen
  2. Conversion of protoporphyrin IX to Heme
17
Q

Which two enzymes in heme synthesis are inhibited by heavy metal poisoning (eg. lead)

A
  1. Aminolevulinic acid
    dehydratase
  2. Ferrochelatase
18
Q

What are porphyrias

A

Accumulation of porphyrin-like precursors due to disruption in heme synthese

19
Q

How will the urine appear in individuals wit porphyrias

A

Red urine

20
Q

What is the name of the type of porphyrias caused by heavy metal (lead) poisoning

A

Acquired porphyrias

21
Q

Which type of porphyria is the most common

A

Porphyria cutanea tarda

22
Q

Which type of porphyria occurs when there is a disruption of the synthesis hydroxymethylbilane from porphobilinogen

A

Acute Intermittent Porphyria

23
Q

Which porphyria is associated with photosensitivity

A

Porphyria cutanea tarda

24
Q

Which porphyria, in severe cases, is associated with dark wine colored urine if it is left to stand

A

Acute intermittent porphyria

25
Q

Which two metabolic pathways require Vitamin B6 (pyridoxal phosphate)

A

Transamination during amino acid metabolism and during heme synthesis