May 25, 2021
                 La glycine
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                 La glycine
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Häm-Biosynthese
Häm-Biosynthese in Tieren, Pilzen und alpha-Proteobakterien
Decarboxylierende Verknüpfung von Succinyl-CoA und Glycin unter HSCoA-Abspaltung bildet 5-Aminolävulinsäure (5-ALA) durch 5-ALA-Synthase*
Verknüpfung zweier 5-ALA zu Porphobilinogen
Deaminierende Verknüpfung von vier Porphobilinogen
Hydroxylierung zu Hydroxymethylbilan
Dehydratisierende Zyklisierung zu Uroporphoryrinogen III
4fache Decarboxylierung der kurzen Seitenketten zu Coproporphyrinogen III
O2-abh., decarboxylierende Oxidation zweier Seitenketten zu Protoporphyrinogen IX
O2-abh. Oxidation des Grundgerüsts zu Protoporphyrin IX
Ferrochelatase fügt Eisen-(II)-Ion ein zum Häm (Protohäm; Häm b)
Regulation:
5-ALA-Synthase allosterisch durch Häm gehemmt; translationell durch Eisen gefördert
Häm hemmt Eisenaufnahme und 5-ALA-Synthase-Transport in Mitochondrien
Erkrankungen: Porphyrien
Par Nature