Protein: Uroporphyrinogen decarboxylase (UPD) (URO-D) (EC 4.1.1.37) (P06132)
Organism: Homo sapiens (Human)
Function: Catalyzes the sequential decarboxylation of the four acetate side chains of uroporphyrinogen to form coproporphyrinogen and participates in the fifth step in the heme biosynthetic pathway. Isomer I or isomer III of uroporphyrinogen may serve as substrate, but only coproporphyrinogen III can ultimately be converted to heme. In vitro also decarboxylates pentacarboxylate porphyrinogen I.
Subcellular location: Cytoplasm, cytosol {ECO:0000250|UniProtKB:P70697}.
Sequence: MEANGLGPQGFPELKNDTFLRAAWGEETDYTPVWCMRQAGRYLPEFRETRAAQDFFSTCRSPEACCELTLQPLRRFPLDAAIIFSDILVVPQALGMEVTMVPGKGPSFPEPLREEQDLERLRDPEVVASELGYVFQAITLTRQRLAGRVPLIGFAGAPWTLMTYMVEGGGSSTMAQAKRWLYQRPQASHQLLRILTDALVPYLVGQVVAGAQALQLFESHAGHLGPQLFNKFALPYIRDVAKQVKARLREAGLAPVPMIIFAKDGHFALEELAQAGYEVVGLDWTVAPKKARECVGKTVTLQGNLDPCALYASEEEIGQLVKQMLDDFGPHRYIANLGHGLYPDMDPEHVGAFVDAVHKHSRLLRQN
InterPro domains: - IPR038071: UROD/MetE-like superfamily (homologous_superfamily) [1-367]
- IPR006361: Uroporphyrinogen decarboxylase HemE (family) [16-361]
- IPR000257: Uroporphyrinogen decarboxylase (URO-D) (domain) [15-360]
Interaction partners: - Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
- Protoporphyrinogen oxidase
- Uroporphyrinogen-III synthase
- Porphobilinogen deaminase
- Delta-aminolevulinic acid dehydratase
- Ferrochelatase, mitochondrial
- Hereditary hemochromatosis protein
- 5-aminolevulinate synthase, non-specific, mitochondrial
- Molybdenum cofactor biosynthesis protein 1
- 5-aminolevulinate synthase, erythroid-specific, mitochondrial
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0003824 (catalytic activity), GO:0005515 (protein binding), GO:0016829 (lyase activity), GO:0016830 (carbon-carbon lyase activity), GO:0042802 (identical protein binding), GO:0046983 (protein dimerization activity), GO:0042803 (protein homodimerization activity), GO:0016831 (carboxy-lyase activity)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0009987 (cellular process), GO:0009058 (biosynthetic process), GO:0044237 (cellular metabolic process), GO:0044281 (small molecule metabolic process), GO:0042440 (pigment metabolic process), GO:0006807 (nitrogen compound metabolic process), GO:0071704 (organic substance metabolic process), GO:1901360 (organic cyclic compound metabolic process), GO:1901564 (organonitrogen compound metabolic process), GO:1901576 (organic substance biosynthetic process), GO:0044283 (small molecule biosynthetic process), GO:0046483 (heterocycle metabolic process), GO:0006082 (organic acid metabolic process), GO:0046148 (pigment biosynthetic process), GO:0044249 (cellular biosynthetic process), GO:0006725 (cellular aromatic compound metabolic process), GO:0034641 (cellular nitrogen compound metabolic process), GO:0043603 (amide metabolic process), GO:0042168 (heme metabolic process), GO:1901566 (organonitrogen compound biosynthetic process), GO:1901362 (organic cyclic compound biosynthetic process), GO:0006536 (glutamate metabolic process), GO:0043436 (oxoacid metabolic process), GO:0016053 (organic acid biosynthetic process), GO:0019438 (aromatic compound biosynthetic process), GO:0018130 (heterocycle biosynthetic process), GO:0044271 (cellular nitrogen compound biosynthetic process), GO:0033013 (tetrapyrrole metabolic process), GO:0006783 (heme biosynthetic process), GO:0006778 (porphyrin-containing compound metabolic process), GO:0019752 (carboxylic acid metabolic process), GO:0033014 (tetrapyrrole biosynthetic process), GO:0009064 (glutamine family amino acid metabolic process), GO:0046394 (carboxylic acid biosynthetic process), GO:0043648 (dicarboxylic acid metabolic process), GO:0006779 (porphyrin-containing compound biosynthetic process)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005829 (cytosol), GO:0005737 (cytoplasm), GO:0031974 (membrane-enclosed lumen), GO:0005654 (nucleoplasm), GO:0043233 (organelle lumen), GO:0043229 (intracellular organelle), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle), GO:0070013 (intracellular organelle lumen), GO:0031981 (nuclear lumen), GO:0005634 (nucleus)