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Leptins Lecture > Synthesis of Heme > Flashcards

Synthesis of Heme Flashcards

1
Q

Porphyrins are synthesized by

A

glycine and succinyl Co-A

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2
Q

Succinyl Co-A + glycine —->

A

delta-aminolevulinate

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3
Q

alpha aminolevulinate —>

catalyzed by whom?

A

4-Porphobilinogen

ALA-dehydratase

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4
Q

4- porphobilinogen —>

catalyzed by whom

A

linear tetrapyrrole

porphobilinogen deaminase

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5
Q

linear tetrapyrrole —>

catalyzed by whom

A

uroporphyrinogen III

Uroporphyrinogen III synthase (and cosynthase)

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6
Q

Uroporphyrinogen III —>

A

Coproporphyrinogen III

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7
Q

Acute intermittent porphyria

 ?

effects what reactions?

A
  • affects heme synthesis
  • caused by deficiency of porphobilinogen deaminase
  • characterized by build up of porphobilinogen
  • hepatic form of porphyria
    symptoms: abdominal pain

LOW HEME PRODUCTION AND HIGH PRECURSOR PRODUCTION

prevents catalysis of 4 porphobilinogen into linear tetrapyrrole

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8
Q

Erythropoietic porphryia

A

caused by deficiency of cosynthase, coenzyme to uroporphyrinogen III synthase

causes by build up uroporphyrinogen I

causes hemolytic anemia, photosensitivity

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9
Q

ALA synthase regulation

A

inhibited/regulated by hemin

requires coenzyme PLP-B6

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10
Q

What are the causes of porphyrias?

A

Genetic environmental defects in enzymes

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11
Q

Tyrosine —>

A

Thyroxine

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12
Q

Tyrosine —>

A

Epinephrine

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13
Q

Tryptophan —>

A

Serotonin

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14
Q

Tryptophan —>

A

Nicotinamide unit of NAD

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15
Q

Serine –>

A

amino unit of sphingosine

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16
Q

Histidine—>

17
Q

Congenital erythropoietic porphyria

A

prematurely destroyes erythrocytes, cause by insufficient cosynthase,

probably occurs in the bone if they ask

18
Q

where does acute intermittent porphyria occur?

19
Q

over production of alpha-aminolevulinate characterizes what disease?

A

acute intermittent porphyria

20
Q

production of urophorphyrinogen 1 characterizes what disease

A

Congenital erythropoietic porphyria

Leptins Lecture (7 decks)

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