Page 47 - Color Atlas Of Pathophysiology (S Silbernagl Et Al, Thieme 2000)

A. Defects of Hemoglobin Synthesis
Glycine Succinyl-CoA Synthesis

α-ALA synthase Gene defect
1
δ-Aminolevulinic
acid (δ-ALA) Hereditary sidero-
blastic anemia
Fe Transferrin
Protoporphyrine
Iron deficiency etc. Anemias: Disorders of Hemoglobin
(see next page)

Iron deficiency
N N anemia
Fe
N N
Plate 3.5
Globin synthesis
Heme
Gene defects
Gene defect
2 α-chains
Heme 2 β-chains 5 Glu 7
Hemoglobin A
deficiency 2
5 Val 7

1 146 1 141 α-Thalassemia
(with Hbγ 4 ; Hbβ 4 ) 6
Hemoglobin A 1 (= Hbα 2 β 2 )
β α β-Thalassemia False β-chain
(with Hb F=Hbα 2 γ 2
and Hb A 2 =Hbα 2 δ 2 )
Hemoglobin S

α β

Sickle
erythrocyte
Normal
erythrocyte
Sickle-cell anemia 37

Silbernagl/Lang, Color Atlas of Pathophysiology © 2000 Thieme
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