Page 265 - Color Atlas Of Pathophysiology (S Silbernagl Et Al, Thieme 2000)

A. Abnormalities of Heme Synthesis

Succinyl-CoA
+ glycine
Sideroblastic anemia
δ-ALA-
synthase-2 1
Circular
Fe incorporation (blue)
α-amino- in erythroblasts
β-ketoadipate
δ-amino- Synthesis, Porphyrias
levulinate in urine
δ-ALA-dehydratase deficiency
δ-ALA-
dehydratase 2
Lead
Porphobili- in urine Heme
nogen (PBG)
Acute intermittent porphyria Neurological and
PBG psychogenic
desaminase 3 disorders Plate 8.7
Hydroxy- Uroporphyrinogen I in urine
methylbilane Hemolytic
Congenital erythropoietic porphyria anemia,
Uroporphyrinogen- 4 red teeth
III-cosynthase
Uroporphy- in urine
rinogen III
Porphyria cutanea tarda
Uroporphyrinogen
decarboxylase 5 Photo: G.Krämer
Copropor- in urine
phyrinogen III in stool
Hereditary coproporphyria
Coproporphyrino-
gen-III-oxidase 6
Protopor- in stool
phyrinogen
Porphyria variegata
Protoporphyrino-
gen oxidase 7 Marked
photosensitivity
Protopor-
phyrin in stool
+ Fe
Protoporphyria
Ferrochelatase 8
Lead
Heme Hemoglobin and other heme proteins 255

Silbernagl/Lang, Color Atlas of Pathophysiology © 2000 Thieme
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