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uroporphyrinogen iii and Porphyrias

uroporphyrinogen iii has been researched along with Porphyrias in 31 studies

Uroporphyrinogens: Porphyrinogens which are intermediates in heme biosynthesis. They have four acetic acid and four propionic acid side chains attached to the pyrrole rings. Uroporphyrinogen I and III are formed from polypyrryl methane in the presence of uroporphyrinogen III cosynthetase and uroporphyrin I synthetase, respectively. They can yield uroporphyrins by autooxidation or coproporphyrinogens by decarboxylation.

Porphyrias: A diverse group of metabolic diseases characterized by errors in the biosynthetic pathway of HEME in the LIVER, the BONE MARROW, or both. They are classified by the deficiency of specific enzymes, the tissue site of enzyme defect, or the clinical features that include neurological (acute) or cutaneous (skin lesions). Porphyrias can be hereditary or acquired as a result of toxicity to the hepatic or erythropoietic marrow tissues.

Research Excerpts

ExcerptRelevanceReference
" This work examines whether the four sequential decarboxylations occur at the same active site, and explores whether hexachlorobenzene-induced porphyria affects the behavior of the enzyme."7.73Abnormal kinetic behavior of uroporphyrinogen decarboxylase obtained from rats with hexachlorobenzene-induced porphyria. ( Cárdenas, ML; Chaufan, G; Corvi, MM; Ríos de Molina, Mdel C; San Martín de Viale, LC, 2005)
" Qualitative and quantitative studies of the porphyrins and the porphyrinogen carboxylyase of the liver, spleen, kidney, harderian gland and erythrocytes from normal rats and from those hexachlorobenzene-induced porphyria were carried out."7.65Porphyrins and porphyrinogen carboxy-lase in hexachlorobenzene-induced porphyria. ( De Calmanovici, RW; Ríos De Molina, MD; San Martín De Viale, LC; Tomio, JM, 1977)
" This work examines whether the four sequential decarboxylations occur at the same active site, and explores whether hexachlorobenzene-induced porphyria affects the behavior of the enzyme."3.73Abnormal kinetic behavior of uroporphyrinogen decarboxylase obtained from rats with hexachlorobenzene-induced porphyria. ( Cárdenas, ML; Chaufan, G; Corvi, MM; Ríos de Molina, Mdel C; San Martín de Viale, LC, 2005)
"The porphyrias are a heterogeneous group of clinical disorders that share a common etiologic background in that each manifests a major metabolic defect in the synthesis of heme."3.67Porphyria. Basic science aspects. ( Bickers, DR, 1986)
"The excretion of position isomers of type III porphyrins, not only in porphyric disorders in which the enzymatic decarboxylation of uroporphyrinogen III to coproporphyrinogen III is known to or suspected to play a role, but also in normal man and in conditions like erythropoietic protoporphyria and lead-intoxication, in which no such changes have ever been reported, or would be expected for theoretical reasons, has been described."3.66Urinary excretion of position isomers type III in normal man and a series of different types of porphyrias. ( Eriksen, L, 1980)
" Qualitative and quantitative studies of the porphyrins and the porphyrinogen carboxylyase of the liver, spleen, kidney, harderian gland and erythrocytes from normal rats and from those hexachlorobenzene-induced porphyria were carried out."3.65Porphyrins and porphyrinogen carboxy-lase in hexachlorobenzene-induced porphyria. ( De Calmanovici, RW; Ríos De Molina, MD; San Martín De Viale, LC; Tomio, JM, 1977)
"Uroporphyria was produced by injecting animals with iron-dextran and giving the porphyrin precursor 5-aminolaevulinic acid in the drinking water."1.30Uroporphyria produced in mice by iron and 5-aminolaevulinic acid does not occur in Cyp1a2(-/-) null mutant mice. ( Bement, WJ; Dalton, T; Gorman, N; Nebert, DW; Sinclair, JF; Sinclair, PR; Smith, AG; Walton, HS, 1998)
"The porphyria is not dependent on pretreatment with iron and persists for at least 21 days after withdrawal of ALA."1.27Uroporphyria produced in mice by 20-methylcholanthrene and 5-aminolaevulinic acid. ( Bement, WJ; Elder, GH; Gorman, N; Lambrecht, RW; Roberts, AG; Sinclair, JA; Sinclair, PR; Urquhart, AJ, 1988)

Research

Studies (31)

TimeframeStudies, this research(%)All Research%
pre-199022 (70.97)18.7374
1990's7 (22.58)18.2507
2000's2 (6.45)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Nakano, K1
Ishizuka, M1
Sakamoto, KQ1
Fujita, S1
Chaufan, G1
Corvi, MM1
San Martín de Viale, LC5
Cárdenas, ML1
Ríos de Molina, Mdel C1
Cantoni, L1
Dal Fiume, D1
Ruggieri, R1
Kondo, M1
Urata, G1
Shimizu, Y1
Kostrzewska, E2
Gregor, A2
de Verneuil, H1
Grandchamp, B2
Nordmann, Y2
Eriksen, L1
Miller, DM1
Woods, JS1
Smith, AG3
Francis, JE2
Sinclair, PR2
Gorman, N2
Dalton, T1
Walton, HS1
Bement, WJ2
Sinclair, JF1
Nebert, DW1
de Catabbi, SB1
de Calmanovici, RW2
Minutolo, C1
Aldonatti, C1
Jackson, AH1
Sancovich, HA1
Ferramola, AM1
Evans, N1
Games, DE1
Matlin, SA1
Elder, GH2
Smith, SG1
Brugsch, J1
Müller, H1
Gidari, AS1
Levere, RD1
Walsh, JR1
Granick, S1
Beale, SI1
Traczyk, A1
Rudowski, W1
Ríos De Molina, MD1
Tomio, JM1
Moore, MR1
Thompson, GG1
Goldberg, A1
Ippen, H1
Seubert, A1
Seubert, S1
Herrero, C1
Piñol Aguade, J1
Lecha, M1
Muniesa, MA1
Almeida, J1
Mallolas, J1
Kushner, JP1
Steinmuller, DP1
Lee, GR1
Billi de Catabbi, S1
Ríos de Molina, MC2
Luo, JL1
Lim, CK1
Beukeveld, GJ1
Wolthers, BG1
Deybach, JC1
Wadman, SK1
Lannfelt, L1
Wetterberg, L1
Gellerfors, P1
Lilius, L1
Floderus, Y1
Thunell, S1
De Matteis, F1
Harvey, C1
Reed, C1
Hempenius, R1
Urquhart, AJ1
Roberts, AG1
Lambrecht, RW1
Sinclair, JA1
Wainstok de Calmanovici, R1
Mukerji, SK1
Pimstone, NR1
Bickers, DR1

Reviews

3 reviews available for uroporphyrinogen iii and Porphyrias

ArticleYear
Enzymatic formation and cellular regulation of heme synthesis.
    Seminars in hematology, 1977, Volume: 14, Issue:2

    Topics: 5-Aminolevulinate Synthetase; Aminolevulinic Acid; Animals; Coenzyme A; Coproporphyrinogens; Enzyme

1977
Hepatic porphyrias. Current concepts.
    Postgraduate medicine, 1977, Volume: 62, Issue:2

    Topics: 5-Aminolevulinate Synthetase; Acute Disease; Coproporphyrinogens; Female; Genetic Diseases, Inborn;

1977
Hemes, chlorophylls, and related compounds: biosynthesis and metabolic regulation.
    Advances in enzymology and related areas of molecular biology, 1978, Volume: 46

    Topics: 5-Aminolevulinate Synthetase; Aminolevulinic Acid; Animals; Bacteria; Chlorophyll; Fungi; Heme; Hemi

1978

Other Studies

28 other studies available for uroporphyrinogen iii and Porphyrias

ArticleYear
Absolute requirement for iron in the development of chemically induced uroporphyria in mice treated with 3-methylcholanthrene and 5-aminolevulinate.
    Biometals : an international journal on the role of metal ions in biology, biochemistry, and medicine, 2009, Volume: 22, Issue:2

    Topics: Aminolevulinic Acid; Animals; Cytochrome P-450 CYP1A2; Iron; Male; Methylcholanthrene; Mice; Mice, I

2009
Abnormal kinetic behavior of uroporphyrinogen decarboxylase obtained from rats with hexachlorobenzene-induced porphyria.
    Journal of biochemical and molecular toxicology, 2005, Volume: 19, Issue:1

    Topics: Animals; Female; Hexachlorobenzene; Kinetics; Liver; Porphyrias; Porphyrinogens; Rats; Rats, Wistar;

2005
Decarboxylation of uroporphyrinogen I and III in 2,3,7,8-tetrachlorodibenzo-p-dioxin induced porphyria in mice.
    The International journal of biochemistry, 1984, Volume: 16, Issue:5

    Topics: Animals; Carboxy-Lyases; Decarboxylation; Dioxins; Erythrocytes; Liver; Male; Mice; Mice, Inbred C57

1984
Decreased liver delta-aminolaevulinate dehydratase activity in porphyria cutanea tarda and in alcoholism.
    Clinical science (London, England : 1979), 1983, Volume: 65, Issue:4

    Topics: Adult; Alcoholism; Ferrous Compounds; Hot Temperature; Humans; Liver; Liver Extracts; Male; Middle A

1983
[Normal activity of uroporphyrinogen I synthase during an attack of acute intermittent porphyria].
    Polski tygodnik lekarski (Warsaw, Poland : 1960), 1980, Nov-17, Volume: 35, Issue:46

    Topics: Acute Disease; Adult; Ammonia-Lyases; Erythrocytes; Female; Humans; Hydroxymethylbilane Synthase; Po

1980
Some kinetic properties of human red cell uroporphyrinogen decarboxylase.
    Biochimica et biophysica acta, 1980, Jan-11, Volume: 611, Issue:1

    Topics: Carboxy-Lyases; Erythrocytes; Humans; Kinetics; Porphyrias; Structure-Activity Relationship; Substra

1980
Urinary excretion of position isomers type III in normal man and a series of different types of porphyrias.
    Scandinavian journal of clinical and laboratory investigation, 1980, Volume: 40, Issue:1

    Topics: Animals; Chromatography, Paper; Coproporphyrinogens; Decarboxylation; Female; Humans; Isomerism; Mal

1980
Redox activities of mercury-thiol complexes: implications for mercury-induced porphyria and toxicity.
    Chemico-biological interactions, 1993, Volume: 88, Issue:1

    Topics: Drug Interactions; Glutathione; Hydrogen Peroxide; In Vitro Techniques; Mercury; Oxidation-Reduction

1993
Genetic variation of iron-induced uroporphyria in mice.
    The Biochemical journal, 1993, Apr-01, Volume: 291 ( Pt 1)

    Topics: Aminolevulinic Acid; Animals; Drug Tolerance; Hydrocarbons; Iron; Liver; Mice; Mice, Hairless; Mice,

1993
Uroporphyria produced in mice by iron and 5-aminolaevulinic acid does not occur in Cyp1a2(-/-) null mutant mice.
    The Biochemical journal, 1998, Feb-15, Volume: 330 ( Pt 1)

    Topics: Aminolevulinic Acid; Animals; Cytochrome P-450 CYP1A1; Cytochrome P-450 CYP1A2; Cytochrome P-450 Enz

1998
Porphyria-induced hepatic porphyrinogen carboxy-lyase inhibitor and its interaction with the active site(s) of the enzyme.
    Biochemistry and molecular biology international, 1999, Volume: 47, Issue:6

    Topics: Animals; Arginine; Binding Sites; Carboxy-Lyases; Enzyme Inhibitors; Female; Hexachlorobenzene; Huma

1999
Macrocyclic intermediates in the biosynthesis of porphyrins.
    Philosophical transactions of the Royal Society of London. Series B, Biological sciences, 1976, Feb-05, Volume: 273, Issue:924

    Topics: Animals; Chickens; Coproporphyrinogens; Hexachlorobenzene; Humans; Porphyrias; Porphyrins; Protoporp

1976
[Urinary excretion of porphyrins and their precursors. Longterm observations in acute intermittent porphyrias (author's transl)].
    MMW, Munchener medizinische Wochenschrift, 1979, Mar-30, Volume: 121, Issue:13

    Topics: Aminolevulinic Acid; Coproporphyrins; Estrogens; Humans; Porphobilinogen; Porphyrias; Porphyrins; Ur

1979
[Diagnosis of acute intermittent porphyria on the basis of uroporphyrinogen I synthase activity in the erythrocytes; comparison of 2 methods].
    Polski tygodnik lekarski (Warsaw, Poland : 1960), 1979, Aug-13, Volume: 34, Issue:33

    Topics: Adolescent; Adult; Ammonia-Lyases; Child; Child, Preschool; Erythrocytes; Evaluation Studies as Topi

1979
Porphyrins and porphyrinogen carboxy-lase in hexachlorobenzene-induced porphyria.
    The Biochemical journal, 1977, Dec-15, Volume: 168, Issue:3

    Topics: Animals; Carboxy-Lyases; Chromatography, Gel; Female; Hexachlorobenzene; Porphyrias; Porphyrins; Rat

1977
The biosynthesis of haem in congenital (erythropoietic) porphyria.
    The International journal of biochemistry, 1978, Volume: 9, Issue:12

    Topics: 5-Aminolevulinate Synthetase; Adult; Child; Child, Preschool; Coproporphyrinogens; Erythrocytes; Ery

1978
In vitro studies of the metabolic pathway from 8-COOH porphyrins to 4-COOH porphyrins.
    Dermatosen in Beruf und Umwelt. Occupation and environment, 1978, Volume: 26, Issue:3

    Topics: Coproporphyrinogens; Decarboxylation; Fibroblasts; Humans; Porphyrias; Porphyrinogens; Porphyrins; U

1978
The role of iron in the pathogenesis of porphyria cutanea tarda. II. Inhibition of uroporphyrinogen decarboxylase.
    The Journal of clinical investigation, 1975, Volume: 56, Issue:3

    Topics: Animals; Carboxy-Lyases; Chromatography, Paper; Depression, Chemical; In Vitro Techniques; Iron; Liv

1975
Studies on the active centre of rat liver porphyrinogen carboxylase in vivo effect of hexachlorobenzene.
    The International journal of biochemistry, 1991, Volume: 23, Issue:7-8

    Topics: Animals; Arginine; Binding Sites; Carboxy-Lyases; Diethyl Pyrocarbonate; Epoxy Compounds; Female; He

1991
Random decarboxylation of uroporphyrinogen III by human hepatic uroporphyrinogen decarboxylase.
    Journal of chromatography, 1991, May-31, Volume: 566, Issue:2

    Topics: Chromatography, High Pressure Liquid; Decarboxylation; Humans; Isomerism; Liver; Porphyrias; Skin Di

1991
A retrospective study of a patient with homozygous form of acute intermittent porphyria.
    Journal of inherited metabolic disease, 1990, Volume: 13, Issue:5

    Topics: Acute Disease; Aminolevulinic Acid; Child, Preschool; Chromatography, High Pressure Liquid; Erythroc

1990
Mutations in acute intermittent porphyria detected by ELISA measurement of porphobilinogen deaminase.
    Journal of clinical chemistry and clinical biochemistry. Zeitschrift fur klinische Chemie und klinische Biochemie, 1989, Volume: 27, Issue:11

    Topics: Ammonia-Lyases; Cross Reactions; Enzyme-Linked Immunosorbent Assay; Erythrocytes; Female; Heterozygo

1989
Oxidation of uroporphyrinogens by hydroxyl radicals. Evidence for nonporphyrin products as potential inhibitors of uroporphyrinogen decarboxylase.
    FEBS letters, 1988, Jun-20, Volume: 233, Issue:2

    Topics: Animals; Carbon Radioisotopes; Carboxy-Lyases; Free Radicals; Humans; Hydroxides; Hydroxyl Radical;

1988
Increased oxidation of uroporphyrinogen by an inducible liver microsomal system. Possible relevance to drug-induced uroporphyria.
    The Biochemical journal, 1988, Feb-15, Volume: 250, Issue:1

    Topics: Aminolevulinic Acid; Animals; Benzoflavones; Cells, Cultured; Chick Embryo; Cytochrome P-450 CYP1A1;

1988
Uroporphyria produced in mice by 20-methylcholanthrene and 5-aminolaevulinic acid.
    The Biochemical journal, 1988, Jul-15, Volume: 253, Issue:2

    Topics: Aminolevulinic Acid; Animals; Cytochrome P-450 CYP1A1; Cytochrome P-450 Enzyme System; Hexachloroben

1988
Liver porphyrinogen carboxylase in hexachlorobenzene porphyric rats. Studies with intermediate porphyrinogens of series III and with uroporphyrinogen I.
    The International journal of biochemistry, 1987, Volume: 19, Issue:4

    Topics: Animals; Carboxy-Lyases; Chlorobenzenes; Female; Hexachlorobenzene; Liver; Porphyrias; Porphyrinogen

1987
In vitro studies of the mechanism of inhibition of rat liver uroporphyrinogen decarboxylase activity by ferrous iron under anaerobic conditions.
    Archives of biochemistry and biophysics, 1986, Feb-01, Volume: 244, Issue:2

    Topics: Anaerobiosis; Animals; Buffers; Carboxy-Lyases; Decarboxylation; Ferrous Compounds; In Vitro Techniq

1986
Porphyria. Basic science aspects.
    Dermatologic clinics, 1986, Volume: 4, Issue:2

    Topics: 5-Aminolevulinate Synthetase; Bone Marrow; Complement Activation; Heme; Humans; Lipid Metabolism; Li

1986