uridine 5' monophosphate [UMP] synthase; orotate phosphoribosyltransferase & orotate 5'-phosphate decarboxylase (UMPS)

Function

• pyrimidine metabolism; UMP biosynthesis via de novo
• pathway; UMP from HCO3-: step 5/6
• pyrimidine metabolism; UMP biosynthesis via de novo
• pathway; UMP from HCO3-: step 6/6

orotidine 5'-phosphate + diphosphate
         <-->
orotate + 5-phospho-alpha-D-ribose 1-diphosphate

orotidine 5'-phosphate <--> UMP + CO2

Structure

• homodimer
• purine/pyrimidine phosphoribosyltransferase family
• decarboxylase family

Alternative splicing

named isoforms=2

Pathology

• defects in UMPS are a cause of hereditary orotic aciduria (HOA) [MIM:258900]; also known as orotic aciduria type 1, a recessive disease characterized by retarded growth, anemia, & excessive urinary excretion of orotic acid

Laboratory

• uridine monophosphate synthetase in erythrocytes

More general terms

• carboxy-lyase or decarboxylase
• orotate phosphoribosyltransferase

Database

• UniProt: http://www.uniprot.org/uniprot/P11172.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=7372
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:7372
• OMIM: https://mirror.omim.org/entry/258900