Uroporphyrinogen decarboxylase

Uroporphyrinogen decarboxylase
	Surface model in complex with coproporphyrinogen according to PDB 1R3Q
	Existing structural data : 1j93 , 1jph , 1jpi , 1jpk , 1r3q , 1r3r , 1r3s , 1r3t , 1r3u , 1r3v , 1r3w , 1r3y , 1uro , 2eja , 2inf , 2q6z , 2q71
Properties of human protein
Mass / length primary structure	367 AS ; 40.8 kDa
Secondary to quaternary structure	Homodimer
Identifier
Gene names	UROD URO-D; UPD
External IDs	OMIM : 176100 ; UniProt : P06132 ; MGI : 98916 ;
Enzyme classification
EC, category	4.1.1.37 , lyase
Response type	Decarboxylation
Substrate	Uroporphyrinogen III
Products	Coproporphyrinogen + 4 CO 2
Occurrence
Homology family	Uroporphyrinogen decarboxylase
Parent taxon	Creature
	Orthologue

Uroporphyrinogen carboxylase (UROD) is the name of the enzyme that removes four carboxy groups from uroporphyrinogen III ; this reaction step is important for the biosynthesis of porphyrin in the metabolism of all living things . Porphyrin is the starting material for heme , vitamin B ₁₂ and other cofactors in humans , for chlorophyll in plants and also plays a role in bacterial metabolism . A deficiency or a change in the UROD enzyme in certain places can lead to a metabolic disease in humans , porphyria cutanea tarda . The cause of the deficiency is always a mutation of the UROD gene .

biosynthesis

The UROD gene is located on chromosome 1 in humans and its 10 exons extend over 3,430 base pairs. The transcript is 1,300 bases long and after translation and post-translational modification finally results in the UROD enzyme with 367 amino acids .

${\ displaystyle \ longrightarrow}$ + 4 CO ₂

Uroporphyrinogen III is decarboxylated four times to give coproporphyrinogen III. It is noteworthy that the reaction does not require any cofactors .

Wikibooks: Biochemistry and Pathobiochemistry: Porphyrin Biosynthesis - Learning and Teaching Materials