Heme Flashcards
does protoporphyrin III have iron?
No
iron is incorporated via ferrochelotase, resulting in HEME
cofacctor of ALA synthase
pyridoxine
glycine + succinyl coa
inhibited by lead
ALA dehydratase
ferrochelatase
lead poisoning:
lead deposits in
epiphyses
other name for uroporphyrinogen I synthase
PBG deaminase
HMB synthase
(hydroxymethylbilane; linear)
remarks on uroporphyrinogn III synthase
cyclization
AP-AP-AP-PA
only one with AR mutation
werewolves
4 congenital erythropoietic porphyria
only AR
uroporphyrinogen III synthase
defect in porphyria cutanea tarda
#5: uroporphyrinogen decarboxylase inc urinary uroporphyrin I
most common symptom of AIP causing admission
abdominal pain
examples of acute porphyria
3,6,7,2
6: Coprophyrinogn oxidase
7: protophorphyrinogen oxidase
examples of cutaneous porphyria
5,8,4
8: ferrochelatase
“protoporphyria”
“erythropoietic protoporphyria”
most common in children
8 eruthropoietic protoporphyria
ferrochelatase
3rd m/c in aadults
erythropoietic disorders (not hepatic)
1,4,8
1: ala synthase
4: uroporphyrinogen III synthasae
8: ferro chelatase
mutation in ala synthase leads to
X-linked sideroblastic anemia
not a porphyria
variegate porphyria
7: protopophyrinogen oxidase
