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5- aminolevulinate synthase, nonspecific, mitochondrial Delta- aminolevulinic acid dehydratase Porphobilinogen deaminase Uroporphyrinogen- III synthase Uroporphyrinogen decarboxylase Uroporphyrinogen decarboxylase Oxygen- dependent coproporphyrinogen- III oxidase, mitochondrial Ferritin, mitochondrial Ferrochelatase, mitochondrial Protoheme IX farnesyltransferase, mitochondrial Cytochrome c oxidase assembly protein COX15 homolog Heme oxygenase 1 Biliverdin reductase A UDP- glucuronosyltransferase 2B17 Beta- glucuronidase Protoporphyrinogen oxidase Glycine Accumulation Coproporphyrinogen III Accumulation Coproporphyrinogen III Heme Heme Protoporphyrin IX Protoporphyrin IX Succinyl-CoA Accumulation 5- Aminolevulinic acid CO2 Accumulation Porphobilinogen H2O H2O Hydroxymethylbilane NH3 Uroporphyrinogen III H2O Uroporphyrin III Uroporphyrinogen I Coproporphyrinogen I CO2 Coproporphyrin I Uroporphyrin I CO2 Coproporphyrin III O2 H+ Protoporphyrinogen IX CO2 H2O Fe2+ H+ O2 Fe3+ H2O H+ Farnesyl pyrophosphate Heme O Heme A O2 Biliverdin Fe2+ Carbon monoxide H2O NAD Bilirubin NADH Uridine diphosphate glucuronic acid Bilirubin diglucuronide Uridine 5'-diphosphate H2O Urobilinogen O2 H2O2 Pyridoxal 5'-phosphate Zinc 2Fe-2S Zinc FAD AH2 A Alcohol Glycine and Serine Metabolism Intracellular Space Mitochondria Outer mitochondrial membrane Inner mitochondrial membrane Microsome Microsome Lysosome
Mitochondria Alas1 Alad Hmbs Uros Urod Urod Cpox Ftmt Fech Cox10 Cox15 Hmox1 Blvra Ugt2b17 Gusb Ppox Glycine Accumulation Coproporphyrinogen III Accumulation Coproporphyrinogen III Heme Heme Protoporphyrin IX Protoporphyrin IX Succinyl-CoA Accumulation 5- Aminolevulinic acid Carbon dioxide Accumulation Porphobilinogen Water Water Hydroxymethylbilane Ammonia Uroporphyrinogen III Water Uroporphyrin III Uroporphyrinogen I Coproporphyrinogen I Carbon dioxide Coproporphyrin I Uroporphyrin I Carbon dioxide Coproporphyrin III Oxygen Hydrogen Protoporphyrinogen IX Carbon dioxide Water Fe2+ Hydrogen Oxygen Fe3+ Water Hydrogen Farnesyl pyrophosphate Heme O Heme A Oxygen Biliverdin Fe2+ Carbon monoxide Water NAD Bilirubin NADH Uridine diphosphate glucuronic acid Bilirubin diglucuronide Uridine 5'-diphosphate Water Urobilinogen Oxygen Hydrogen peroxide AH2 A Alcohol Glycine and Serine Metabolism
Alas1 Alad Hmbs Uros Urod Urod Cpox Ftmt Fech Cox10 Cox15 Hmox1 Blvra Ugt2b17 Gusb Ppox Gly Accumulation Coprgn3 Accumulation Coprgn3 Heme Heme PrtopIX PrtopIX Suc-CoA Accumulation 5-Amnva CO2 Accumulation PBG H2O H2O HMBilan NH3 Urppr3 H2O Urop3 Uropg1 CPPgen CO2 CopropI Urop1 CO2 CoprIII O2 H+ Protop9 CO2 H2O Fe2+ H+ O2 Fe3+ H2O H+ FarPP Heme O Heme A O2 Bilvdn Fe2+ CO H2O NAD BilRubn NADH UDPGlcA BRubnDG UDP H2O Urbilgn O2 H2O2 Pyr-5'P Zinc 2Fe2S Zinc FAD AH2 A Alcohol PW000157 Intracellular Space Mitochondria Outer mitochondrial membrane Inner mitochondrial membrane Microsome Microsome Lysosome
Mitochondria Alas1 Alad Hmbs Uros Urod Urod Cpox Ftmt Fech Cox10 Cox15 Hmox1 Blvra Ugt2b17 Gusb Ppox Gly Accumulation Coprgn3 Accumulation Coprgn3 Heme Heme PrtopIX PrtopIX Suc-CoA Accumulation 5-Amnva CO2 Accumulation PBG H2O H2O HMBilan NH3 Urppr3 H2O Urop3 Uropg1 CPPgen CO2 CopropI Urop1 CO2 CoprIII O2 H2 Protop9 CO2 H2O Fe2+ H2 O2 Fe3+ H2O H2 FarPP Heme O Heme A O2 Bilvdn Fe2+ CO H2O NAD BilRubn NADH UDPGlcA BRubnDG UDP H2O Urbilgn O2 H2O2 AH2 A Alcohol PW000157