Porphyria cutanea tarda and hepatoerythropoietic porphyria: Identification of 19 novel uroporphyrinogen III decarboxylase mutations [PDF]
Molecular Genetics and Metabolism, 2019 Porphyria Cutanea Tarda (PCT) is a cutaneous porphyria that results from the hepatic inhibition of the heme biosynthetic enzyme uroporphyrinogen decarboxylase (UROD), and can occur either in the absence or presence of an inherited heterozygous UROD mutation (PCT subtypes 1 and 2, respectively).
Yedidyah Weiss +5 more
europepmc +7 more sources
Order of uroporphyrinogen III decarboxylation on incubation of porphobilinogen and uroporphyrinogen III with erythrocyte uroporphyrinogen decarboxylase [PDF]
Biochemical Journal, 1993 The isomeric compositions of the heptacarboxylic, hexacarboxylic and pentacarboxylic porphyrinogens formed by incubation of porphobilinogen with human red-cell haemolysates have been analysed and compared with those derived from incubation with chemically prepared uroporphyrinogen III as substrate.
J, Luo, C K, Lim
semanticscholar +5 more sources
Decarboxylation of uroporphyrinogen III by erythrocyte uroporphyrinogen decarboxylase. Evidence for a random decarboxylation mechanism [PDF]
Biochemical Journal, 1990 The isomeric composition of type-III heptacarboxylic porphyrinogens derived from decarbosylation of uroporphyrinogen III by erythrocyte uroporphyringogen decarboxylase was analysed by h.p.l.c. with electrochemical detection. All four possible isomers were identified, and there were little differences in the proportion of isomers formed by erythrocytes ...
J, Luo, C K, Lim
semanticscholar +5 more sources
Crystal Structure and Substrate Binding Modeling of the Uroporphyrinogen-III Decarboxylase from Nicotiana tabacum [PDF]
Journal of Biological Chemistry, 2001 The enzymatic catalysis of many biological processes of life is supported by the presence of cofactors and prosthetic groups originating from the common tetrapyrrole precursor uroporphyrinogen-III.
Berta M. Martins +4 more
semanticscholar +4 more sources
Coexistence of Deficiencies of Uroporphyrinogen III Synthase and Decarboxylase in a Patient with Congenital Erythropoietic Porphyria and in His Family [PDF]
cclm, 1997 A hitherto undescribed dual deficiency of uroporphyrinogen III synthase and uroporphyrinogen decarboxylase was observed in the erythrocytes in a 14 year-old patient who had presented with congenital erythropoietic porphyria since early childhood. Whereas congenital erythropoietic porphyria was metabolically and clinically overt, a hereditary deficiency
Freesemann, Anne G. +2 more
semanticscholar +6 more sources
Investigations of rat liver uroporphyrinogen decarboxylase. Comparisons of porphyrinogens I and III as substrates and the inhibition by porphyrins [PDF]
Biochemical Journal, 1981 1. The decarboxylations of uroporphyrinogens, hepta-, hexa- and penta-carboxyporphyrinogens I and III by porphyrinogen carboxy-lyase (EC 4.1.1.37) in rat liver supernatant have been compared as functions of substrate concentrations. Although Km and Vmax.
A G, Smith, J E, Francis
semanticscholar +5 more sources
Direct Assay of Enzymes in Heme Biosynthesis for the Detection of Porphyrias by Tandem Mass Spectrometry. Uroporphyrinogen Decarboxylase and Coproporphyrinogen III Oxidase [PDF]
Analytical Chemistry, 2008 We report new assays of enzymes uroporphyrinogen decarboxylase (UROD) and coproporphyrinogen III oxidase (CPO) in the heme biosynthetic pathway. The assays were developed for use in clinical diagnostics of inherited disorders porphyria cutanea tarda and hereditary coproporphyria, respectively. Electrospray ionization tandem mass spectrometry is used to
Yuesong, Wang +5 more
semanticscholar +4 more sources
Action of uroporphyrinogen decarboxylase on uroporphyrinogen III [PDF]
Biochemical Journal, 1991 J L, Luo, C K, Lim
semanticscholar +5 more sources
Action of uroporphyrinogen decarboxylase on uroporphyrinogen-III: a reassessment of the clockwise decarboxylation hypothesis [PDF]
Biochemical Journal, 1991 Timothy D. Lash
semanticscholar +5 more sources
Journal of Biological Chemistry, 1983 H, de Verneuil, S, Sassa, A, Kappas
semanticscholar +4 more sources