beta-ureidopropionase (beta-alanine synthase, N-carbamoyl-beta-alanine amidohydrolase, BUP-1, UPB1, BUP1)

Function

• converts N-carbamyl-beta-aminoisobutyric acid to beta-aminoisobutyric acid & N-carbamyl-beta-alanine to beta-alanine, NH3 & CO2
• allosteric enzyme with positive cooperativity toward substrate N-carbamoyl-beta-alanine
• amino-acid biosynthesis, beta-alanine biosynthesis

N-carbamoyl-beta-alanine + H2O <--> beta-alanine + CO2 + NH3

Cofactor: binds 1 Zn+2 per subunit

Structure

• belongs to the CN hydrolase family, BUP subfamily
• contains 1 CN hydrolase domain

Compartment

cytoplasm

Pathology

• defects in UPB1 are the cause of beta-ureidopropionase deficiency

More general terms

• hydrolase
• metalloprotein

Additional terms

• beta-ureidopropionase deficiency

Database

• OMIM: https://mirror.omim.org/entry/606673
• UniProt: http://www.uniprot.org/uniprot/Q9UBR1.html