Protein Family Models

This is a family of bacterial putative uroporphyrinogen-III C-methyltransferase proteins. It forms one of the members of a complex of proteins involved in the biogenesis of the inner membrane in E.coli. Uroporphorphyrin-III C-methyltransferase (HemX) is a single spanning inner membrane protein that regulates the activity of NAD(P)H:glutamyl-tRNA reductase (HemA) in the tetrapyrrole biosynthesis pathway [1,2]. [1]. 3062586. Nucleotide sequence of the hemX gene, the third member of the Uro operon of Escherichia coli K12. Sasarman A, Echelard Y, Letowski J, Tardif D, Drolet M;. Nucleic Acids Res. 1988;16:11835. [2]. 16079137. Protein complexes of the Escherichia coli cell envelope. Stenberg F, Chovanec P, Maslen SL, Robinson CV, Ilag LL, von Heijne G, Daley DO;. J Biol Chem. 2005;280:34409-34419. (from Pfam)

Date:

2024-10-16

This family consists of uroporphyrinogen-III synthase HemD EC:4.2.1.75 also known as Hydroxymethylbilane hydrolyase (cyclizing) from eukaryotes, bacteria and archaea. This enzyme catalyses the reaction: Hydroxymethylbilane <=> uroporphyrinogen-III + H(2)O. Some members of this family are multi-functional proteins possessing other enzyme activities related to porphyrin biosynthesis, such as Swiss:Q59294 with Pfam:PF00590, however the aligned region corresponds with the uroporphyrinogen-III synthase EC:4.2.1.75 activity only. Uroporphyrinogen-III synthase is the fourth enzyme in the heme pathway [2]. Mutant forms of the Uroporphyrinogen-III synthase gene cause congenital erythropoietic porphyria in humans a recessive inborn error of metabolism also known as Gunther disease [1]. [1]. 8829650. Molecular basis of congenital erythropoietic porphyria: mutations in the human uroporphyrinogen III synthase gene. Xu W, Astrin KH, Desnick RJ;. Hum Mutat 1996;7:187-192. [2]. 7597845. Isolation of the gene HEM4 encoding uroporphyrinogen III synthase in Saccharomyces cerevisiae. Amillet JM, Labbe-Bois R;. Yeast 1995;11:419-424. (from Pfam)

Date:

2024-10-16

uroporphyrinogen-III synthase catalyzes cyclization of the linear tetrapyrrole, hydroxymethylbilane, to the macrocyclic uroporphyrinogen III

Date:

2017-02-03