Page last updated: 2024-10-06

Hereditary Coproporphyria (HCP)

Proteins (16)

ProteinSynonymsTaxonomy
UDP-glucuronosyltransferase 2B11UDPGT 2B11; EC 2.4.1.17Homo sapiens (human)
Porphobilinogen deaminasePBG-D; EC 2.5.1.61; Hydroxymethylbilane synthase; HMBS; Pre-uroporphyrinogen synthaseHomo 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)
Heme oxygenase 1HO-1; EC 1.14.14.18Homo 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)
Beta-glucuronidaseEC 3.2.1.31; Beta-G1Homo sapiens (human)
Ferritin, mitochondrialEC 1.16.3.1Homo 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)
Biliverdin reductase ABVR A; EC 1.3.1.24; Biliverdin-IX alpha-reductaseHomo sapiens (human)
Cytochrome c oxidase assembly protein COX15 homologHomo sapiens (human)
Feline leukemia virus subgroup C receptor-related protein 2Calcium-chelate transporter; CCTHomo sapiens (human)

Compounds (22)

CompoundDescription
aminolevulinic acidA compound produced from succinyl-CoA and GLYCINE as an intermediate in heme synthesis. It is used as a PHOTOCHEMOTHERAPY for actinic KERATOSIS.
ammonium hydroxideThe hydroxy salt of ammonium ion. It is formed when AMMONIA reacts with water molecules in solution.
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)
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.
NADH
uridine diphosphateA uracil nucleotide containing a pyrophosphate group esterified to C5 of the sugar moiety.
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.
uroporphyrin III
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.
porphobilinogen
uroporphyrinogen iii
protoporphyrinogen
uridine diphosphate glucuronic acidA nucleoside diphosphate sugar which serves as a source of glucuronic acid for polysaccharide biosynthesis. It may also be epimerized to UDP iduronic acid, which donates iduronic acid to polysaccharides. In animals, UDP glucuronic acid is used for formation of many glucosiduronides with various aglycones.
coproporphyrinogen iii
farnesyl pyrophosphatea sesquiterpene that dimerizes to SQUALENE; RN given refers to cpd without isomeric designation
bilirubinA bile pigment that is a degradation product of HEME.
NADA coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
hydroxymethylbilanetetrapyrrole intermediate in pigment biosynthesis by Euglena gracilis; universal intermediate in biosynthesis of uroporphyrinogen III; structure given in first source