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5- Aminolevulinate inner mitochondrial membrane transporter 5- Aminolevulinate outer mitochondrial membrane transporter Coproporphyrinogen III mitochondrial outer membrane transporter Heme mitochondrial outer membrane transporter 5-aminolevulinate synthase, nonspecific, mitochondrial Porphobilinogen deaminase Uroporphyrinogen- III synthase Uroporphyrinogen decarboxylase Coproporphyrinogen- III oxidase, mitochondrial Protoporphyrinogen oxidase Ferrochelatase, mitochondrial Methylmalonyl-CoA mutase, mitochondrial Methylmalonyl-CoA epimerase, mitochondrial Hemoglobin subunit alpha Hemoglobin subunit beta Hemoglobin subunit alpha Hemoglobin subunit beta Hemoglobin subunit alpha Hemoglobin subunit beta Delta- aminolevulinic acid dehydratase S- adenosylmethionine synthase isoform type-2 Methionine adenosyltransferase 2 subunit beta Methionine synthase cytosolic Propionyl-CoA 5-Aminolevulinic acid 5-Aminolevulinic acid 5-Aminolevulinic acid Coproporphyrinogen III Coproporphyrinogen III Heme Heme Glycine Succinyl-CoA CoA CO2 Porphobilinogen H2O Hydroxymethylbilane NH3 Uroporphyrinogen III H2O CO2 O2 Protoporphyrinogen IX CO2 H2O O2 Protoporphyrin IX H2O2 Fe2+ H+ R- Methylmalonyl- CoA S- Methylmalonyl- CoA ATP HCO3- ADP Pi H2O L-Methionine H2O ATP S- Adenosylmethionine Diphosphoric acid Pi Homocysteine 5- Methyltetrahydrofolic acid Tetrahydrofolic acid Pyridoxal 5'-phosphate FAD 2Fe-2S Oxygen Hydrogen Nitrenium ion Carbamate Zinc Magnesium Potassium Protein degradation Fat metabolism Propionyl-CoA carboxylase alpha chain, mitochondrial Propionyl-CoA carboxylase beta chain, mitochondrial dipyrromethane Adenosylcobalamin Biotin Magnesium Zinc (II) ion Methylcobalamin Inner membrane Outer membrane Intermembrane space Vitamin B12 is a cofactor for methylmalonyl-CoA mutase. Lack of vitamin B12 leads to accumulation of R-Methylmalonyl CoA that is believed to be responsible for the neurological manifestations of B12 deficiency Methylmalonyl-CoA is responsible for producing succinyl-CoA which is an essential part of hemoglobin synthesis Mitochondria S-adenosylmethionine is a methyl donor for nearly 100 substrates, comprised of DNA, RNA, hormones, proteins, as well as lipids Tetrahydrofolate is needed to maintain normal erythropoiesis. Lack of vitamin B12 causes interruption of this reaction and leading to megaloblastic anemia. Vitamin B12 is a cofactor for methionine synthase
Mitochondria Unknown Unknown Unknown Unknown ALAS1 HMBS UROS UROD CPOX PPOX FECH MUT MCEE HBA1 HBB HBA1 HBB HBA1 HBB ALAD MAT2A MAT2B MTR Propionyl-CoA 5-Aminolevulinic acid 5-Aminolevulinic acid 5-Aminolevulinic acid Coproporphyrinogen III Coproporphyrinogen III Heme Heme Glycine Succinyl-CoA Coenzyme A Carbon dioxide Porphobilinogen Water Hydroxymethylbilane Ammonia Uroporphyrinogen III Water Carbon dioxide Oxygen Protoporphyrinogen IX Carbon dioxide Water Oxygen Protoporphyrin IX Hydrogen peroxide Fe2+ Hydrogen Ion R- Methylmalonyl- CoA S- Methylmalonyl- CoA Adenosine triphosphate Hydrogen carbonate Adenosine diphosphate Phosphate Water L-Methionine Water Adenosine triphosphate S- Adenosylmethionine Diphosphoric acid Phosphate Homocysteine 5- Methyltetrahydrofolic acid Tetrahydrofolic acid Protein degradation Fat metabolism PCCA PCCB