uroporphyrinogen decarboxylase; UPD (UROD)

Function

• catalyzes decarboxylation of four acetate groups of uroporphyrinogen-III to yield coproporphyrinogen-III
• porphyrin metabolism; protoporphyrin-IX biosynthesis
• coproporphyrinogen-III from 5-aminolevulinate: step 4/4

uroporphyrinogen III = coproporphyrinogen + 4 CO2

Structure

• homodimer
• belongs to the uroporphyrinogen decarboxylase family

Compartment

cytoplasm

Pathology

• defects in UROD are the cause of
  • familial porphyria cutanea tarda
  • hepatoerythropoietic porphyria

More general terms

• carboxy-lyase or decarboxylase
• phosphoprotein

Additional terms

• erythropoietic protoporphyria (EPP)
• heme synthesis
• porphyria cutanea tarda (PCT)

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=7389
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:7389
• OMIM: https://mirror.omim.org/entry/176100
• OMIM: https://mirror.omim.org/entry/613521
• UniProt: http://www.uniprot.org/uniprot/P06132.html