Page last updated: 2024-10-06

Porphyrin metabolism

Proteins (20)

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)
Multidrug resistance-associated protein 1 EC 7.6.2.2; ATP-binding cassette sub-family C member 1; Glutathione-S-conjugate-translocating ATPase ABCC1; 7.6.2.3; Leukotriene C(4) transporter; LTC4 transporterHomo sapiens (human)
Canalicular multispecific organic anion transporter 1ATP-binding cassette sub-family C member 2; Canalicular multidrug resistance protein; Multidrug resistance-associated protein 2; 7.6.2.2Homo sapiens (human)
Broad substrate specificity ATP-binding cassette transporter ABCG2EC 7.6.2.2; ATP-binding cassette sub-family G member 2; Breast cancer resistance protein; CDw338; Mitoxantrone resistance-associated protein; Placenta-specific ATP-binding cassette transporter; Urate exporterHomo sapiens (human)
Solute carrier organic anion transporter family member 1B1Liver-specific organic anion transporter 1; LST-1; OATP-C; Sodium-independent organic anion-transporting polypeptide 2; OATP-2; Solute carrier family 21 member 6Homo sapiens (human)
Uroporphyrinogen decarboxylaseUPD; URO-D; EC 4.1.1.37Homo sapiens (human)
AlbuminHomo 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)
UDP-glucuronosyltransferase 1A1 UGT1A1; EC 2.4.1.17; Bilirubin-specific UDPGT isozyme 1; hUG-BR1; UDP-glucuronosyltransferase 1-1; UDPGT 1-1; UGT1*1; UGT1-01; UGT1.1; UDP-glucuronosyltransferase 1A isoform 1Homo sapiens (human)
Protoporphyrinogen oxidasePPO; EC 1.3.3.4Homo sapiens (human)
Solute carrier organic anion transporter family member 1B3Liver-specific organic anion transporter 2; LST-2; Organic anion transporter 8; Organic anion-transporting polypeptide 8; OATP-8; Solute carrier family 21 member 8Homo sapiens (human)
Uroporphyrinogen-III synthaseUROIIIS; UROS; EC 4.2.1.75; Hydroxymethylbilane hydrolyase [cyclizing]; Uroporphyrinogen-III cosynthaseHomo sapiens (human)
UDP-glucuronosyltransferase 1A4UGT1A4; EC 2.4.1.17; Bilirubin-specific UDPGT isozyme 2; hUG-BR2; UDP-glucuronosyltransferase 1-4; UDPGT 1-4; UGT1*4; UGT1-04; UGT1.4; UDP-glucuronosyltransferase 1-D; UGT-1D; UGT1DHomo 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)
Protoheme IX farnesyltransferase, mitochondrialEC 2.5.1.141; Heme O synthaseHomo sapiens (human)
Cytochrome c oxidase assembly protein COX15 homologHomo sapiens (human)
Beta-galactosidase Clostridium perfringens

Compounds (34)

CompoundDescription
hydronium ion
Ammonium
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.
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
carbon monoxideCarbon monoxide (CO). A poisonous colorless, odorless, tasteless gas. It combines with hemoglobin to form carboxyhemoglobin, which has no oxygen carrying capacity. The resultant oxygen deprivation causes headache, dizziness, decreased pulse and respiratory rates, unconsciousness, and death. (From Merck Index, 11th ed)
ZincA metallic element of atomic number 30 and atomic weight 65.38. It is a necessary trace element in the diet, forming an essential part of many enzymes, and playing an important role in protein synthesis and in cell division. Zinc deficiency is associated with ANEMIA, short stature, HYPOGONADISM, impaired WOUND HEALING, and geophagia. It is known by the symbol Zn.
Orthophosphate
leadA soft, grayish metal with poisonous salts; atomic number 82, atomic weight 207.2, symbol Pb.
uridine diphosphateA uracil nucleotide containing a pyrophosphate group esterified to C5 of the sugar moiety.
adenosine diphosphateAdenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
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.
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.
succinyl-coenzyme A
preuroporphyrinogen
uroporphyrinogen iii
protoporphyrinogen
dipyrromethanestructure in first source
heme Ohemes of cytochrome o; heme A-like molecule, containing a 17-carbon hydroxyethylfarnesyl side chain but with a methyl residue replacing the formyl group
NADPNicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5'-phosphate (NMN) coupled by pyrophosphate linkage to the 5'-phosphate adenosine 2',5'-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed)
NADPNicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5'-phosphate (NMN) coupled by pyrophosphate linkage to the 5'-phosphate adenosine 2',5'-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed)
coproporphyrinogen iii
farnesyl pyrophosphatea sesquiterpene that dimerizes to SQUALENE; RN given refers to cpd without isomeric designation
protoporphyrin ixRN given refers to parent cpd; structure in Merck Index, 9th ed, #7685
adenosine triphosphateAn adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.
stercobilinRN given refers to (2R,3R,4S,16S,17R,18R)-isomer
urobilin
Porphobilinogen
BilirubinA bile pigment that is a degradation product of HEME.
Coenzyme A
Biliverdine1,3,6,7-Tetramethyl-4,5-dicarboxyethyl-2,8-divinylbilenone. Biosynthesized from hemoglobin as a precursor of bilirubin. Occurs in the bile of AMPHIBIANS and of birds, but not in normal human bile or serum.
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.
creolinfrom refined coal tar oils