Page last updated: 2024-10-06

superpathway of b heme biosynthesis from glycine

Proteins (10)

ProteinSynonymsTaxonomy
Porphobilinogen deaminasePBG-D; EC 2.5.1.61; Hydroxymethylbilane synthase; HMBS; Pre-uroporphyrinogen synthaseHomo sapiens (human)
5-aminolevulinate synthase, erythroid-specific, mitochondrialALAS-E; EC 2.3.1.37; 5-aminolevulinic acid synthase 2; Delta-ALA synthase 2; Delta-aminolevulinate synthase 2Homo sapiens (human)
Ferrochelatase, mitochondrialEC 4.99.1.1; Heme synthase; Protoheme ferro-lyaseHomo sapiens (human)
Uroporphyrinogen decarboxylaseUPD; URO-D; EC 4.1.1.37Homo sapiens (human)
Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrialCOX; Coprogen oxidase; Coproporphyrinogenase; EC 1.3.3.3Homo sapiens (human)
Delta-aminolevulinic acid dehydrataseALADH; EC 4.2.1.24; Porphobilinogen synthaseHomo sapiens (human)
Protoporphyrinogen oxidasePPO; EC 1.3.3.4Homo sapiens (human)
Uroporphyrinogen-III synthaseUROIIIS; UROS; EC 4.2.1.75; Hydroxymethylbilane hydrolyase [cyclizing]; Uroporphyrinogen-III cosynthaseHomo sapiens (human)
5-aminolevulinate synthase, nonspecific, mitochondrialALAS-H; EC 2.3.1.37; 5-aminolevulinic acid synthase 1; Delta-ALA synthase 1; Delta-aminolevulinate synthase 1Homo sapiens (human)
5-aminolevulinate synthase 2EC 2.3.1.37; 5-aminolevulinic acid synthase; Delta-ALA synthase; Delta-aminolevulinate synthaseCereibacter sphaeroides 2.4.1

Compounds (36)

CompoundDescription
hydronium ion
pyridoxalThe 4-carboxyaldehyde form of VITAMIN B 6 which is converted to PYRIDOXAL PHOSPHATE which is a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid.
RubidiumAn element that is an alkali metal. It has an atomic symbol Rb, atomic number 37, and atomic weight 85.47. It is used as a chemical reagent and in the manufacture of photoelectric cells.
Ammonium
copper sulfateA sulfate salt of copper. It is a potent emetic and is used as an antidote for poisoning by phosphorus. It also can be used to prevent the growth of algae.
MercuryA silver metallic element that exists as a liquid at room temperature. It has the atomic symbol Hg (from hydrargyrum, liquid silver), atomic number 80, and atomic weight 200.59. Mercury is used in many industrial applications and its salts have been employed therapeutically as purgatives, antisyphilitics, disinfectants, and astringents. It can be absorbed through the skin and mucous membranes which leads to MERCURY POISONING. Because of its toxicity, the clinical use of mercury and mercurials is diminishing.
CopperA heavy metal trace element with the atomic symbol Cu, atomic number 29, and atomic weight 63.55.
ironA metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN.
ManganeseA trace element with atomic symbol Mn, atomic number 25, and atomic weight 54.94. It is concentrated in cell mitochondria, mostly in the pituitary gland, liver, pancreas, kidney, and bone, influences the synthesis of mucopolysaccharides, stimulates hepatic synthesis of cholesterol and fatty acids, and is a cofactor in many enzymes, including arginase and alkaline phosphatase in the liver. (From AMA Drug Evaluations Annual 1992, p2035)
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
LithiumAn element in the alkali metals family. It has the atomic symbol Li, atomic number 3, and atomic weight [6.938; 6.997]. Salts of lithium are used in treating BIPOLAR DISORDER.
Hydrogen PeroxideA strong oxidizing agent used in aqueous solution as a ripening agent, bleach, and topical anti-infective. It is relatively unstable and solutions deteriorate over time unless stabilized by the addition of acetanilide or similar organic materials.
iodineA nonmetallic element of the halogen group that is represented by the atomic symbol I, atomic number 53, and atomic weight of 126.90. It is a nutritionally essential element, especially important in thyroid hormone synthesis. In solution, it has anti-infective properties and is used topically.
potassiumAn element in the alkali group of metals with an atomic symbol K, atomic number 19, and atomic weight 39.10. It is the chief cation in the intracellular fluid of muscle and other cells. Potassium ion is a strong electrolyte that plays a significant role in the regulation of fluid volume and maintenance of the WATER-ELECTROLYTE BALANCE.
MagnesiumA metallic element that has the atomic symbol Mg, atomic number 12, and atomic weight 24.31. It is important for the activity of many enzymes, especially those involved in OXIDATIVE PHOSPHORYLATION.
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
OxygenAn element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.
uroporphyrin III
succinyl-coenzyme A
preuroporphyrinogen
iodoacetamideAn alkylating sulfhydryl reagent. Its actions are similar to those of iodoacetate.
phenylglyoxalA reagent that is highly selective for the modification of arginyl residues. It is used to selectively inhibit various enzymes and acts as an energy transfer inhibitor in photophosphorylation.
diethyl pyrocarbonatePreservative for wines, soft drinks, and fruit juices and a gentle esterifying agent.
ethylmaleimideA sulfhydryl reagent that is widely used in experimental biochemical studies.
diacetylCarrier of aroma of butter, vinegar, coffee, and other foods.
Porphobilinogen
hydrogen cyanideHydrogen cyanide (HCN); A toxic liquid or colorless gas. It is found in the smoke of various tobacco products and released by combustion of nitrogen-containing organic materials.
coproporphyrin IRN given refers to cpd with specified locants; see also record for coproporphyrin III; zinc coproporphyrin I is fluorescent and a characteristic component of meconium
coproporphyrin IIIRN given refers to unlabeled parent cpd; see also record for coproporphyrin I
protoporphyrinogen
coproporphyrinogen III
Coenzyme A
EthacridineA topically applied anti-infective agent.
Flavin-Adenine DinucleotideA condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972)
hemeThe color-furnishing portion of hemoglobin. It is found free in tissues and as the prosthetic group in many hemeproteins.
Haematoporphyrin IX