Porphyria cutanea tarda, or the uroporphyrinogen decarboxylase deficiency diseases

The Metabolic Basis of Inherited Disease.

1983 • 2,885 citations

Stereospecific, high affinity binding of 2,3,7,8-tetrachlorodibenzo-p-dioxin by hepatic cytosol. Evidence that the binding species is receptor for induction of aryl hydrocarbon hydroxylase.

1976 • 1,343 citations

The replacement of the nondescript term ‘heavy metals’ by a biologically and chemically significant classification of metal ions

1980 • 1,150 citations

Purification and characterization of liver microsomal cytochromes P-450: electrophoretic, spectral, catalytic, and immunochemical properties and inducibility of eight isozymes isolated from rats treated with phenobarbital or .beta.-naphthoflavone

1982 • 1,021 citations

Effect of ethanol on liver 8-aminolaevulinate synthetase in rats.

1968 • 430 citations

Cytoplasmic vacuolation of mouse peritoneal macrophages and the uptake into lysosomes of weakly basic substances.

1981 • 424 citations

Serum Ferritin in Patients with Iron Overload and with Acute and Chronic Liver Diseases

1975 • 344 citations

Halogenated Biphenyls, Terphenyls, Naphthalenes, Dibenzodioxins and Related Products

1982 • 275 citations

DEANOL IN SPONTANEOUS AND INDUCED DYSKINESIAS

1975 • 254 citations

Genetic Expression of Aryl Hydrocarbon Hydroxylase by 2,3,7,8-Tetrachlorodibenzo-p-dioxin: Evidence for a Receptor Mutation in Genetically Non-responsive Mice

1975 • 238 citations

The Role of Iron in Enzymatic Lipid Peroxidation

1982 • 233 citations

Porphyrins in Human Diseases.

1977 • 225 citations

Cutaneous Porphyria in Turkey

1960 • 220 citations

Industrially Acquired Porphyria

1964 • 210 citations

Decreased Activity of Hepatic Uroporphyrinogen Decarboxylase in Sporadic Porphyria Cutanea Tarda

1978 • 204 citations

PORPHYRIN CONTENT OF BONE MARROW AND LIVER IN THE VARIOUS FORMS OF PORPHYRIA

1954 • 198 citations

Familial and sporadic porphyria cutanea

1978 • 181 citations

STUDIES ON THE CHEMOTHERAPY OF THE HUMAN MALARIAS. VI. THE PHYSIOLOGICAL DISPOSITION, ANTIMALARIAL ACTIVITY, AND TOXICITY OF SEVERAL DERIVATIVES OF 4-AMINOQUINOLINE 12

1948 • 180 citations

Culturing hepatocytes and other differentiated cells

1984 • 151 citations

REDUCED ACTIVE THYROID-HORMONE LEVELS IN ACUTE ILLNESS

1976 • 142 citations

2,3,7,8-Tetrachlorodibenzo- p -dioxin: A Potent Inducer of δ-Aminolevulinic Acid Synthetase

1973 • 140 citations

Macrocyclic intermediates in the biosynthesis of porphyrins

1976 • 139 citations

Purification and properties of uroporphyrinogen decarboxylase from human erythrocytes. A single enzyme catalyzing the four sequential decarboxylations of uroporphyrinogens I and III.

1983 • 138 citations

The role of iron in the pathogenesis of porphyria cutanea tarda. II. Inhibition of uroporphyrinogen decarboxylase.

1975 • 132 citations

HEPATOERYTHROPOIETIC PORPHYRIA: A NEW UROPORPHYRINOGEN DECARBOXYLASE DEFECT OR HOMOZYGOUS PORPHYRIA CUTANEA TARDA?

1981 • 124 citations

Iron Deficiency Prevents Liver Toxicity of 2,3,7,8-Tetrachlorodibenzo- p -Dioxin

1979 • 110 citations

Effects of pentachlorophenol on hepatic drug-metabolizing enzymes and porphyria related to contamination with chlorinated dibenzo-p-dioxins and dibenzofurans

1977 • 104 citations

The Effect of the Porphyrogenic Compound, Hexachlorobenzene, on the Activity of Hepatic Uroporphyrinogen Decarboxylase in the Rat

1976 • 102 citations

Serum ferritin concentration and bone marrow iron stores: a prospective study.

1978 • 96 citations

Metabolism and effects of hexachlorobenzene on hepatic microsomal enzymes in the rat

1975 • 96 citations

Porphyria Cutanea Tarda

1968 • 95 citations

Experimental porphyria induced in rats by hexachloro-benzene. A study of the porphyrins excreted by urine

1970 • 89 citations

Differentiation of porphyria cutanea tarda symptomatica from other types of porphyria by measurement of isocoproporphyrin in faeces.

1975 • 88 citations

The Role of Iron in the Pathogenesis of Porphyria Cutanea Tarda

1972 • 86 citations

2,3,7,8-Tetrachlorodibenzo-p-dioxin: environmental contaminant and molecular probe.

1976 • 83 citations

Decreased Hepatic Uroporphyrinogen Decarboxylase Activity in Porphyria Cutanea Tarda

1982 • 82 citations

Lipid peroxidation as a possible cause of TCDD toxicity

1983 • 81 citations

Hepatic porphyria induced by 2,3,7,8-tetrachlorodibenzo-p-dioxin in the mouse.

1973 • 79 citations

The Treatment of Porphyria Cutanea Tarda by Venesection

1974 • 78 citations

The Porphyrias: A Story of Inheritance and Environment.

1972 • 73 citations

Delta-aminolaevulinic acid synthetase activity in normal and porphyric human livers.

1967 • 72 citations

Identification of a group of tetracarboxylate porphyrins, containing one acetate and three propionate β-substituents, in faeces from patients with symptomatic cutaneous hepatic porphyria and from rats with porphyria due to hexachlorobenzene

1972 • 69 citations

Porphyrin metabolism in porphyria cutanea tarda.

1977 • 68 citations

Identification of Two Types of Porphyria Cutanea Tarda by Measurement of Erythrocyte Uroporphyrinogen Decarboxylase

1980 • 67 citations

The Porphyrias: A Story of Inheritance and Environment

1964 • 66 citations

THE EFFECT OF REPLENISHMENT OF IRON STORES AFTER PHLEBOTOMY THERAPY IN PORPHYRIA CUTANEA TARDA

1971 • 66 citations

Iron Metabolism in Porphyria Cutanea Tarda and in Erythropoietic Protoporphyria

1973 • 66 citations

Porphyrin Metabolism in Experimental Hepatic Siderosis in the Rat

1972 • 64 citations

IRON STORES IN ALCOHOL ABUSERS

1969 • 63 citations

Studies on the toxicology of hexachlorobenzene

1978 • 63 citations

Immunoreactive uroporphyrinogen decarboxylase is unchanged in porphyria caused by TCDD and hexachlorobenzene

1982 • 62 citations

STUDIES ON LOW DOSE CHLOROQUINE THERAPY AND THE ACTION OF CHLOROQUINE IN SYMPTOMATIC PORPHYRIA

1972 • 61 citations

Frequency of occurrence of hepatocellular carcinoma in patients with porphyria cutanea tarda in long-term follow-up.

1972 • 59 citations

Red-Cell Uroporphyrinogen Decarboxylase Activity in Porphyria Cutanea Tarda and in Other Forms of Porphyria

1978 • 58 citations

EFFECT OF ETHANOL ON LIVER δ-AMINOLÆVULINATE SYNTHETASE IN RATS

1968 • 58 citations

Evidence of chlorodibenzo-p-dioxin and chlorodibenzofuran in hexachlorobenzene

1974 • 57 citations

THE EFFECT OF PHLEBOTOMY THERAPY IN PORPHYRIA CUTANEA TARDA

1971 • 54 citations

The role of iron in the toxicity of 2,3,7,8-tetrachlorodibenzo-(p)-dioxin (TCDD)

1981 • 54 citations

Porphyria cutanea tarda: comparison of cases precipitated by alcohol and estrogens.

1975 • 54 citations

Effect of Ethanol on Liver δ‐Aminolaevulinate Synthetase Activity and Urinary Porphyrin Excretion in Symptomatic Porphyria

1969 • 53 citations

Assessment of the contribution of chlorinated dibenzo-p-dioxins and dibenzofurans to hexachlorobenzene-induced toxicity, porphyria, changes in mixed function oxygenases, and histopathological changes

1978 • 52 citations

The use of thin layer chromatography in the separation of free porphyrins and porphyrin methyl esters

1975 • 46 citations

IMMUNOREACTIVE UROPORPHYRINOGEN DECARBOXYLASE IN PORPHYRIA CUTANEA TARDA

1983 • 42 citations

Quantitation of urinary porphyrins by use of second-derivative spectroscopy.

1979 • 42 citations

Effects of Chloroquine on Patients with Cutaneous Porphyria of the "Symptomatic" Type

1965 • 40 citations

Serum ferritin and iron stores.

1977 • 39 citations

The Porphyrias: Clinical Chemistry, Diagnosis and Methodology

1980 • 37 citations

Hepta-, Hexa-, and Pentacarboxylic Porphyrins of Porphyria Cutanea Tarda

1959 • 36 citations

High-pressure liquid chromatography of plasma free acid porphyrins

1980 • 36 citations

Editorial Retrospective

1983 • 36 citations

Association between induction of aryl hydrocarbon hydroxylase and depression of uroporphyrinogen decarboxylase activity.

1977 • 34 citations

Measurement of Uroporphyrinogen Decarboxylase Using Porphyrinogens Prepared by Chemical Reduction

1982 • 33 citations

The porphyrins of normal human urine, with a comparison of the excretion pattern in porphyria cutanea tarda

1980 • 31 citations

Release of ferrous iron from ferritin by liver microsomes: a possible role in the toxicity of 2,3,7,8-tetrachlorodibenzo-p-dioxin

1984 • 30 citations

Hepatic δ‐Aminolaevulinic Acid Synthetase Activity in Symptomatic Porphyria

1968 • 29 citations

Porphyria Cutanea Tarda

1968 • 29 citations

Biochemical effects of chloroquine therapy in porphyria cutanea tarda

1970 • 29 citations

Uroporphyrinogen decarboxylase. A method for measuring enzyme activity.

1982 • 28 citations

The chromatographic separation of uroporphyrin I and III octamethyl esters

1979 • 27 citations

Measurement of urine porphyrins and porphyrinogens

1976 • 25 citations

A comparative study of the results of phlebotomy therapy and low-dose chloroquine treatment in porphyria cutanea tarda

1981 • 25 citations

Uroporphyrinogen Decarboxylase

1982 • 23 citations

A SURVEY OF PORPHYRIA AMONG PSYCHIATRIC PATIENTS

1972 • 22 citations

Phlebotomy Therapy in Cutaneous Porphyria

1970 • 21 citations

Clinical Removal of Iron

1982 • 21 citations

High-performance liquid chromatography of porphyrin esters

1981 • 21 citations

[Acne chlorina, porphyria cutanea tarda and other manifestations of general poisoning during the manufacture of herbicides. II].

1974 • 20 citations

HEPATIC δ-AMINOLÆVULINIC ACID SYNTHETASE ACTIVITY IN PORPHYRIA CUTANEA TARDA

1972 • 20 citations

Experimental Porphyria Induced in Rats by Hexachlorobenzene

2015 • 20 citations

Hemochromatosis and Red Wine

1967 • 19 citations

THE CLINICAL BIOCHEMISTRY OF THE HUMAN HEPATOCUTANEOUS PORPHYRIAS IN THE LIGHT OF RECENT STUDIES OF NEWLY IDENTIFIED INTERMEDIATES AND PORPHYRIN DERIVATIVES*

1975 • 19 citations

Porphyria cutanea tarda. Remission following chloroquine administration without adverse effects

1968 • 16 citations

Separation of protoporphyrins and related compounds by reversed-phase liquid chromatography.

1979 • 16 citations

Suggested pathobiochemical development of chronic hepatic porphyrias

1971 • 15 citations

Porphyria cutanea tarda and hepatocellular carcinoma

1980 • 15 citations

Isolation of an hydroxylated porphyrin from urine and faeces of patients with cutaneous hepatic porphyria

1970 • 14 citations

Direct spectrofluorophotometric determination of porphyrin in diluted urine.

1976 • 14 citations

RECENT ADVANCES IN THE IDENTIFICATION OF ENZYME DEFICIENCIES IN THE PORPHYRIAS

1983 • 13 citations

Porphyrinanalyse im Lebercylinder bei Porphyria cutanea tarda

1969 • 11 citations

Specimen collection for urinary porphyrin studies

1983 • 11 citations

Stool porphyrins determined by high pressure liquid chromatography and by fractional hydrochloric acid—ether extraction

1979 • 10 citations

Patterns of porphyrin excretion in porphyria cutanea tarda under venesection treatment

1980 • 10 citations

Porphyria cutanea tarda: clinical and laboratory features.

1979 • 10 citations

Location of iron and porphyrin in the liver in porphyria cutanea tarda

1978 • 8 citations

Porphyria cutanea tarda

1982 • 8 citations

Phlebotomy therapy in cutaneous porphyria. Effect on iron and trace metals

1970 • 8 citations

Estrogen-induced porphyria cutanea tarda. Report of three cases

1970 • 8 citations

Chronische hepatische Porphyrie durch polyhalogenisierte aromatische Verbindungen bei Menschen

1978 • 6 citations

Stool porphyrins determined by high pressure liquid chromatography and by fractional hydrochloric acid - ether extraction

1979 • 6 citations

Porphyria Cutanea Tarda Treated by Repeated Venesection: Clinical and Biochemical Response

1969 • 5 citations

A rapid isocratic HPLC procedure for the determination of urinary porphyrins

1980 • 5 citations

Porphyria cutanea tarda.

1972 • 4 citations

Effect of vitamin e on urine porphyrin and steroid profiles in porphyria cutanea tarda; report of four cases

1972 • 3 citations

Porphyrin metabolism in porphyria metabolism in porphyria cutanea tarda.

1962 • 3 citations

Porphyria Cutanea Tarda

1972 • 1 citations

HEPATIC DELTA-AMINOLÆVULINIC-ACID-SYNTHETASE ACTIVITY IN PORPHYRIA CUTANEA TARDA

1972 • 1 citations

Deleted Work

1955 • 0 citations