Any feedback?
Please rate this page
(enzyme.php)
(0/150)

BRENDA support

Disease on EC 4.2.1.24 - porphobilinogen synthase

Please use the Disease Search for a specific query.
Please wait a moment until all data is loaded. This message will disappear when all data is loaded.
DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Adenocarcinoma
Is delta-aminolevulinic acid dehydratase rate limiting in heme biosynthesis following exposure of cells to delta-aminolevulinic acid?
alcohol dehydrogenase deficiency
Ecogenetic and pharmacogenetic studies in Hungary.
Ecogenetic studies in Atacameño Indians.
Alzheimer Disease
Delta-Aminolevulinate dehydratase and glutathione peroxidase activity in Alzheimer's disease: a case-control study.
Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis, lead, and genetic susceptibility: polymorphisms in the delta-aminolevulinic acid dehydratase and vitamin D receptor genes.
Anemia
A role of red cell pyrimidine 5'-nucleotidase in experimental lead poisoning.
Abnormalities of heme biosynthesis in experimental acute renal failure.
Delta-aminolevulinate dehydratase activity in mice with hereditary anemia.
Effects of inorganic lead on delta-aminolevulinic acid dehydratase activity and hematological variables in the rainbow trout, Salmo gairdnerii.
Erythrocyte delta-aminolaevulinic acid dehydratase activity and changes in delta-aminolaevulinic acid concentration in various forms of anaemia.
Erythrocyte delta-aminolevulinic acid dehydrase activity in thalassemia major and sickle-cell anemia.
Heme synthesis in anemia of the uremic state.
Heme synthesis in hereditary hemolytic anemias: decreased delta-aminolevulinic acid synthetase in hemoglobin Köln disease.
Increase in the amount of erythrocyte delta-aminolevulinic acid dehydratase in workers with moderate lead exposure.
Increased erythrocyte uroporphyrinogen-l-synthetase, delta-aminolevulinic acid dehydratase and protoporphyrin in hemolytic anemias.
Iron distribution in Belgrade rat reticulocytes after inhibition of heme synthesis with succinylacetone.
Organic and inorganic forms of selenium inhibited differently fish (Rhamdia quelen) and rat (Rattus norvergicus albinus) delta-aminolevulinate dehydratase.
Oxidative stress and delta-ALA-D activity in chronic renal failure patients.
Reduced aminolevulinic dehydrase activity with increased blood porphyrins in experimental chronic renal failure.
The effect of cadmium on the hematology and on the activity of delta-aminolevulinic acid dehydratase (ALA-D) in blood and hematopoietic tissues of the flounder, Pleuronectes flesus L.
[Delta aminolevulinic acid dehydratase activity in red cells and delta aminolevulinic acid urinary excretion in puerperants with anemia]
Anemia, Aplastic
Benzene inhibition of in vitro rabbit reticulocyte haem synthesis at delta aminolaevulinic acid synthetase: reversal of benzene toxicity by pyridoxine.
Anemia, Hemolytic
Heme synthesis in hereditary hemolytic anemias: decreased delta-aminolevulinic acid synthetase in hemoglobin Köln disease.
Anemia, Hypochromic
A mutation in the gene for delta-aminolevulinic acid dehydratase (ALAD) causes hypochromic anemia in the medaka, Oryzias latipes.
Developmental changes in delta-aminolevulinic acid dehydratase (ALAD) activity and blood reticulocyte percent in the developing rat. A brief note.
Anemia, Iron-Deficiency
Erythrocyte delta-aminolaevulinic acid dehydratase activity and changes in delta-aminolaevulinic acid concentration in various forms of anaemia.
[Changes in the biochemical indices in iron-deficiency erythropoiesis]
Anemia, Megaloblastic
Erythrocyte delta-aminolaevulinic acid dehydratase activity and changes in delta-aminolaevulinic acid concentration in various forms of anaemia.
Anemia, Pernicious
[Porphobilinogen synthase activity and porphyrin biosynthesis in the erythrocytes after incubation with delta-aminolevulinic acid in pernicious anemia patients]
Anemia, Sickle Cell
Heme synthesis in hereditary hemolytic anemias: decreased delta-aminolevulinic acid synthetase in hemoglobin Köln disease.
Arthritis, Rheumatoid
[New aspects in the pathogenesis of anemia in chronic polyarthritis]
Ascorbic Acid Deficiency
Ascorbic acid and cytochrome P-450.
Balkan Nephropathy
Erythrocyte delta-aminolevulinate dehydratase measurements in Balkan endemic nephropathy.
beta-Thalassemia
Erythrocyte delta-aminolevulinic acid dehydrase activity in thalassemia major and sickle-cell anemia.
Heme synthesis in hereditary hemolytic anemias: decreased delta-aminolevulinic acid synthetase in hemoglobin Köln disease.
Brain Neoplasms
Delta-aminolevulinic acid dehydratase polymorphism and risk of brain tumors in adults.
Breast Neoplasms
Downregulation of delta-aminolevulinate dehydratase is associated with poor prognosis in patients with breast cancer.
Effects of Silencing Heme Biosynthesis Enzymes on 5-Aminolevulinic Acid-mediated Protoporphyrin IX Fluorescence and Photodynamic Therapy.
Is delta-aminolevulinic acid dehydratase rate limiting in heme biosynthesis following exposure of cells to delta-aminolevulinic acid?
Carcinogenesis
Axin2+ Peribiliary Glands in the Periampullary Region Generate Biliary Epithelial Stem Cells That Give Rise to Ampullary Carcinoma.
Involvement of oxidative stress in the pre-malignant and malignant states of cervical cancer in women.
Carcinoma
Axin2+ Peribiliary Glands in the Periampullary Region Generate Biliary Epithelial Stem Cells That Give Rise to Ampullary Carcinoma.
Mouse mammary carcinoma delta-aminolevulinate dehydratase.
Carcinoma, Hepatocellular
Heme-biosynthetic enzyme activities and porphyrin accumulation in normal liver and hepatoma cell lines of rat.
Regulation of heme synthesis in HepG2 human hepatoma cells by dimethyl sulfoxide.
Cholangiocarcinoma
Origin and pathological characteristics of Klatskin tumor: a case report and literature review.
Congenital Abnormalities
Sublethal effects of tetrachloro-1,2-benzoquinone--a component in bleachery effluents from pulp mills--on vertebral quality and physiological parameters in fourhorn sculpin.
Coproporphyria, Hereditary
Composition of urinary coproporphyrin isomers I-IV in human porphyrias.
Drugs and acute porphyrias: reasons for a hazardous relationship.
Heme biosynthesis and the porphyrias.
Human hereditary hepatic porphyrias.
Neurovisceral porphyrias: what a hematologist needs to know.
Pathogenesis and clinical features of the acute hepatic porphyrias (AHPs).
Treatment options in acute porphyria, porphyria cutanea tarda, and erythropoietic protoporphyria.
Diabetes Mellitus
Delta-aminolevulinate dehydratase (delta-ALA-D) activity in diabetes and hypothyroidism.
Dietary supplementation of jute leaf (Corchorus olitorius) modulates hepatic delta-aminolevulinic acid dehydratase (?-ALAD) activity and oxidative status in high-fat fed/low streptozotocin-induced diabetic rats.
Enzymic glycation may decrease activity of erythrocytic delta-aminolevulinate dehydratase in diabetes mellitus.
Oxidative stress in mice is dependent on the free glucose content of the diet.
Diabetes Mellitus, Experimental
Delta aminolevulinate dehydratase (ALA-D) activity in human and experimental diabetes mellitus.
Diabetes Mellitus, Type 2
Delta-aminolevulinate dehydratase (delta-ALA-D) activity in diabetes and hypothyroidism.
Peribiliary Glands as a Niche of Extra-Pancreatic Precursors Yielding Insulin-Producing Cells in Experimental and Human Diabetes.
Diabetes, Gestational
Influence of gestational diabetes on the activity of ?-aminolevulinate dehydratase and oxidative stress biomarkers.
Drug-Related Side Effects and Adverse Reactions
Allostery and the dynamic oligomerization of porphobilinogen synthase.
Impact of quaternary structure dynamics on allosteric drug discovery.
Porphobilinogen synthase: An equilibrium of different assemblies in human health.
Essential Tremor
Delta-amino-levulinic acid dehydratase gene and essential tremor.
Fanconi Syndrome
delta-Aminolevulinic acid dehydratase: effects of succinylacetone in rat liver and kidney in an in vivo model of the renal Fanconi syndrome.
Renal heme metabolism in hereditary tyrosinemia: use of succinylacetone in rat renal tubules.
Genetic Diseases, Inborn
Succinylacetone inhibits delta-aminolevulinate dehydratase and potentiates the drug and steroid induction of delta-aminolevulinate synthase in liver.
Glucose Intolerance
Long-term sucrose and glucose consumption decreases the delta-aminolevulinate dehydratase activity in mice.
Hemochromatosis
The relevance of the individual genetic background for the toxicokinetics of two significant neurodevelopmental toxicants: mercury and lead.
Hyperglycemia
A high fat diet inhibits delta-aminolevulinate dehydratase and increases lipid peroxidation in mice (Mus musculus).
Acute diphenyl diselenide treatment reduces hyperglycemia but does not change delta-aminolevulinate dehydratase activity in alloxan-induced diabetes in rats.
Delta-aminolevulinate dehydratase (delta-ALA-D) activity in diabetes and hypothyroidism.
Effect of oral administration of diphenyl diselenide on antioxidant status, and activity of delta aminolevulinic acid dehydratase and isoforms of lactate dehydrogenase, in streptozotocin-induced diabetic rats.
Effects of sub-lethal and chronic lead concentrations on blood and liver ALA-D activity and hematological parameters in Nile tilapia.
Inhibition of delta-aminolevulinate dehydratase is not closely related to the development of hyperglycemia in alloxan-induced diabetic mice.
Long-term sucrose and glucose consumption decreases the delta-aminolevulinate dehydratase activity in mice.
Hypertension
Associations between patella lead and blood pressure in lead workers.
Associations of blood pressure and hypertension with lead dose measures and polymorphisms in the vitamin D receptor and delta-aminolevulinic acid dehydratase genes.
Hypothyroidism
Delta-aminolevulinate dehydratase (delta-ALA-D) activity in diabetes and hypothyroidism.
Experimental hypothyroidism inhibits delta-aminolevulinate dehydratase activity in neonatal rat blood and liver.
Infections
Controlled delivery of antimicrobial gallium ions from phosphate-based glasses.
Intellectual Disability
Increased blood lead levels in mentally retarded children in Greece.
Iron Deficiencies
Erythrocyte delta-aminolaevulinic acid dehydratase activity and changes in delta-aminolaevulinic acid concentration in various forms of anaemia.
Kidney Failure, Chronic
Erythrocyte aminolevulinate dehydratase activity as a lead marker in patients with chronic renal failure.
Inhibition of erythrocyte aminolevulinate dehydratase by a 56.2-kD peptide from uremic plasma.
Oxidative stress and delta-ALA-D activity in chronic renal failure patients.
Leukemia
[Porphobilinogen synthase activity of the erythrocytes in acute leukemia]
Leukemia, Erythroblastic, Acute
delta-Aminolevulinate dehydratase in human erythroleukemia cells: an immunologically distinct enzyme.
Effects of succinylacetone on dimethylsulfoxide-mediated induction of heme pathway enzymes in mouse friend virus-transformed erythroleukemia cells.
Induction of delta-aminolevulinic acid dehydratase in mouse Friend virus-transformed erythroleukemia cells during erythroid differentiation.
Succinylacetone, a potent inhibitor of heme biosynthesis: effect on cell growth, heme content and delta-aminolevulinic acid dehydratase activity of malignant murine erythroleukemia cells.
Liver Cirrhosis
Changes in aminolevulinate synthase and aminolevulinate dehydratase activity in cirrhotic liver.
Liver Diseases
Cloning and expression of the defective genes in delta-aminolevulinate dehydratase porphyria: compound heterozygosity in this hereditary liver disease.
Expression and localization of sterile alpha motif domain containing 5 is associated with cell type and malignancy of biliary tree.
Hepatic heme synthesis in a new model of experimental hemochromatosis: studies in rats fed finely divided elemental iron.
Validity of 5-aminolevulinate dehydratase activity (5-ALAD) for the discrimination of alcoholics and nonalcoholics with chronic liver disease.
Liver Failure
Peribiliary Gland Dilatation in Cirrhosis: Relationship with Liver Failure and Stem Cell/Proliferation Markers.
Lymphoma
[Reduced glutathione and porphobilinogen synthase activity in the erythrocytes of anemic patients with low serum iron]
Malaria
Delta-aminolevulinic acid dehydratase from Plasmodium falciparum: indigenous versus imported.
If there is an effect of lead exposure on malaria, then the activity of delta-aminolevulinate dehydratase (ALAD) may play a role, as ALAD is imported by the parasite from the host.
Malnutrition
Influence of protein calorie malnutrition and fasting on the activities of delta-aminolevulinic acid dehydratase and porphobilinogen deaminase in rats.
Meningioma
Delta-aminolevulinic acid dehydratase polymorphism and risk of brain tumors in adults.
Mesothelioma
Is delta-aminolevulinic acid dehydratase rate limiting in heme biosynthesis following exposure of cells to delta-aminolevulinic acid?
Metabolism, Inborn Errors
Porphobilinogen synthase: An equilibrium of different assemblies in human health.
Multiple Sclerosis
Evaluation of Delta-Aminolevulinic Dehydratase Activity, Oxidative Stress Biomarkers, and Vitamin D Levels in Patients with Multiple Sclerosis.
Neoplasms
Axin2+ Peribiliary Glands in the Periampullary Region Generate Biliary Epithelial Stem Cells That Give Rise to Ampullary Carcinoma.
Is delta-aminolevulinic acid dehydratase rate limiting in heme biosynthesis following exposure of cells to delta-aminolevulinic acid?
Low blood lead levels impair intellectual and hematological function in children from Cartagena, Caribbean coast of Colombia.
Metabolic changes in the heme pathway driven by cyclophosphamide treatment in mice.
Mouse mammary carcinoma delta-aminolevulinate dehydratase.
Peribiliary Glands as the Cellular Origin of Biliary Tract Cancer.
Simultaneous determination of delta-aminolevulinic acid, porphobilinogen, levulinic acid and glycine in culture broth by capillary electrophoresis.
Neurologic Manifestations
Some parameters of haem synthesis in dialysed and non-dialysed uraemic patients.
Obesity
Long-term sucrose and glucose consumption decreases the delta-aminolevulinate dehydratase activity in mice.
Paralysis
New type of acute porphyria with porphobilinogen synthase (delta-aminolevulinic acid dehydratase) defect in the homozygous state.
New type of hepatic porphyria with porphobilinogen synthase defect and intermittent acute clinical manifestation.
Pelger-Huet Anomaly
Impaired heme synthesis in a family with Pelger-Huët anomaly, recurrent abdominal pain attacks and impaired neutrophil motility in vitro.
Photosensitivity Disorders
Is delta-aminolevulinic acid dehydratase rate limiting in heme biosynthesis following exposure of cells to delta-aminolevulinic acid?
porphobilinogen synthase deficiency
5-Aminolevulinic acid dehydratase deficiency porphyria: a twenty-year clinical and biochemical follow-up.
Acute porphyrias: pathogenesis of neurological manifestations.
Association of amino levulinate dehydratase levels and ferrochelatase inhibition in childhood lead exposure.
Clinical Remission of Delta-Aminolevulinic Acid Dehydratase Deficiency through Suppression of Erythroid Heme Synthesis.
Clinically important features of porphyrin and heme metabolism and the porphyrias.
Composition of urinary coproporphyrin isomers I-IV in human porphyrias.
Deficiency of porphobilinogen synthase associated with acute crisis. Diagnosis of the first two cases in Chile by laboratory methods.
delta-Aminolevulinic acid dehydrase (porphobilinogen synthase) in two families with inherited enzyme deficiency.
delta-Aminolevulinic acid dehydratase deficiency can cause delta-aminolevulinate auxotrophy in Escherichia coli.
delta-Aminolevulinic acid dehydratase deficiency porphyria (ADP) with syndrome of inappropriate secretion of antidiuretic hormone (SIADH) in a 69-year-old woman.
Enzymatic defect in a child with hereditary hepatic porphyria due to homozygous delta-aminolevulinic acid dehydratase deficiency: immunochemical studies.
Excretion pattern of faecal coproporphyrin isomers I-IV in human porphyrias.
Heme biosynthesis and the porphyrias.
Hereditary hepatic porphyria due to homozygous delta-aminolevulinic acid dehydratase deficiency: studies in lymphocytes and erythrocytes.
Hereditary hepatic porphyria with delta aminolevulinate dehydrase deficiency: immunologic characterization of the non-catalytic enzyme.
Human hereditary hepatic porphyrias.
Investigations on the formation of urinary coproporphyrin isomers I-IV in 5-aminolevulinic acid dehydratase deficiency porphyria, acute lead intoxication and after oral 5-aminolevulinic acid loading.
Lead poisoning in inherited delta-aminolevulinic acid dehydratase deficiency.
Message amplification phenotyping of an inherited delta-aminolevulinate dehydratase deficiency in a family with acute hepatic porphyria.
Molecular analysis of delta-aminolevulinate dehydratase deficiency in a patient with an unusual late-onset porphyria.
Neurovisceral porphyrias: what a hematologist needs to know.
New type of acute porphyria with porphobilinogen synthase (delta-aminolevulinic acid dehydratase) defect in the homozygous state.
Persistent protoporphyrinemia in hereditary porphobilinogen synthase (delta-aminolevulinic acid dehydrase) deficiency under low lead exposure. A new molecular basis for the pathogenesis of lead intoxication.
Porphobilinogen-synthase (delta-aminolevulinic acid dehydratase) deficiency in bone marrow cells of two patients with porphobilinogen-synthase defect acute porphyria.
Porphyric neuropathy and hereditary delta-aminolevulinic acid dehydratase deficiency in an adult.
The cutaneous porphyrias.
Treatment of the porphyrias.
Treatment options in acute porphyria, porphyria cutanea tarda, and erythropoietic protoporphyria.
Tyrosinemia with aminolevulinic dehydratase deficiency.
[Acute porphyria in familial delta-aminolevulinic acid dehydrase deficiency]
[delta-Aminolevulinate dehydratase deficiency]
[Porphyrin-synthesizing enzymes in erythrocyte]
Porphyria Cutanea Tarda
Erythrocyte delta-aminolevulinic acid dehydratase activity in porphyria cutanea tarda.
Excretion pattern of faecal coproporphyrin isomers I-IV in human porphyrias.
Hepatic heme synthesis in a new model of experimental hemochromatosis: studies in rats fed finely divided elemental iron.
Human hereditary hepatic porphyrias.
In inherited porphyrias, lead intoxication is a toxogenetic disorder.
Study of erythrocyte delta-aminolevulinic acid dehydratase activity in porphyria cutanea tarda.
[Porphyrin-synthesizing enzymes in erythrocyte]
Porphyria, Acute Intermittent
Clinically important features of porphyrin and heme metabolism and the porphyrias.
Drugs and acute porphyrias: reasons for a hazardous relationship.
Excretion pattern of faecal coproporphyrin isomers I-IV in human porphyrias.
Heme biosynthesis and the porphyrias.
Hereditary prophobilinogen synthase deficiency in human associated with acute hepatic porphyria.
Human hereditary hepatic porphyrias.
In inherited porphyrias, lead intoxication is a toxogenetic disorder.
Neurovisceral porphyrias: what a hematologist needs to know.
New type of acute porphyria with porphobilinogen synthase (delta-aminolevulinic acid dehydratase) defect in the homozygous state.
New type of hepatic porphyria with porphobilinogen synthase defect and intermittent acute clinical manifestation.
Pathogenesis and clinical features of the acute hepatic porphyrias (AHPs).
Porphyrin synthesis and mitochondrial respiration in acute intermittent porphyria: studies using cultured human fibroblasts.
The cutaneous porphyrias.
Treatment options in acute porphyria, porphyria cutanea tarda, and erythropoietic protoporphyria.
[Acute porphyria in familial delta-aminolevulinic acid dehydrase deficiency]
[Enzyme deficiency of erythrocytes in human porphyria]
[Porphyrin-synthesizing enzymes in erythrocyte]
[Treatment of acute porphyrias. The importance of follow-up of patients and carriers]
Porphyria, Erythropoietic
In inherited porphyrias, lead intoxication is a toxogenetic disorder.
Late-onset porphyrias: what are they?
[Porphyrin-synthesizing enzymes in erythrocyte]
Porphyria, Variegate
"Glucose effect" and rate limiting function of uroporphyrinogen synthase on porphyrin metabolism in hepatocyte culture: relationship with human acute hepatic porphyrias.
Acute porphyrias: pathogenesis of neurological manifestations.
Composition of urinary coproporphyrin isomers I-IV in human porphyrias.
Drugs and acute porphyrias: reasons for a hazardous relationship.
Enzyme antioxidant defences and oxidative damage in red blood cells of variegate porphyria patients.
Heme biosynthesis and the porphyrias.
Human hereditary hepatic porphyrias.
Neurovisceral porphyrias: what a hematologist needs to know.
Pathogenesis and clinical features of the acute hepatic porphyrias (AHPs).
The "glucose effect" in acute hepatic porphyrias and in experimental porphyria.
Treatment options in acute porphyria, porphyria cutanea tarda, and erythropoietic protoporphyria.
[Drugs and porphyric crisis]
[Treatment of acute porphyrias. The importance of follow-up of patients and carriers]
Porphyrias
5-Aminolevulinate dehydratase porphyria: Update on hepatic 5-aminolevulinic acid synthase induction and long-term response to hemin.
5-Aminolevulinic acid dehydratase deficiency porphyria: a twenty-year clinical and biochemical follow-up.
A comparison of the porphyrin-inducing activity of barbiturates and benzodiazepines in chick embryo liver cells.
Acute porphyrias: pathogenesis of neurological manifestations.
ALAD porphyria is a conformational disease.
Allosteric inhibition of human porphobilinogen synthase.
Clinically important features of porphyrin and heme metabolism and the porphyrias.
Cloning and expression of the defective genes from a patient with delta-aminolevulinate dehydratase porphyria.
Cloning and expression of the defective genes in delta-aminolevulinate dehydratase porphyria: compound heterozygosity in this hereditary liver disease.
Co-synthesis of Human delta-Aminolevulinate Dehydratase (ALAD) Mutants with the Wild-type Enzyme in Cell-free System-Critical Importance of Conformation on Enzyme Activity-.
Composition of urinary coproporphyrin isomers I-IV in human porphyrias.
delta-Aminolevulinate dehydratase (ALAD) porphyria: the first case in North America with two novel ALAD mutations.
delta-Aminolevulinate dehydratase deficient porphyria: identification of the molecular lesions in a severely affected homozygote.
delta-Aminolevulinic acid dehydratase deficiency porphyria (ADP) with syndrome of inappropriate secretion of antidiuretic hormone (SIADH) in a 69-year-old woman.
Direct assay of delta-aminolevulinic acid dehydratase in heme biosynthesis for the detection of porphyrias by tandem mass spectrometry.
Diverse clinical compounds alter the quaternary structure and inhibit the activity of an essential enzyme.
Drugs and acute porphyrias: reasons for a hazardous relationship.
Effects of sulfur-containing metabolites of hexachlorobenzene on the heme metabolic enzymes in rat liver.
Environmental contaminants perturb fragile protein assemblies and inhibit normal protein function.
Erythrocyte delta-aminolevulinic acid dehydratase activity in porphyria cutanea tarda.
Excretion pattern of faecal coproporphyrin isomers I-IV in human porphyrias.
Heme biosynthesis and the porphyrias.
Hepatic heme synthesis in a new model of experimental hemochromatosis: studies in rats fed finely divided elemental iron.
Hereditary prophobilinogen synthase deficiency in human associated with acute hepatic porphyria.
Highly heterogeneous nature of delta-aminolevulinate dehydratase (ALAD) deficiencies in ALAD porphyria.
Human hereditary hepatic porphyrias.
In inherited porphyrias, lead intoxication is a toxogenetic disorder.
Investigations on the formation of urinary coproporphyrin isomers I-IV in 5-aminolevulinic acid dehydratase deficiency porphyria, acute lead intoxication and after oral 5-aminolevulinic acid loading.
Late-onset porphyrias: what are they?
Molecular analysis of delta-aminolevulinate dehydratase deficiency in a patient with an unusual late-onset porphyria.
MORPHEEINS - A NEW PATHWAY FOR ALLOSTERIC DRUG DISCOVERY.
Murine models of the human porphyrias: Contributions toward understanding disease pathogenesis and the development of new therapies.
Neurovisceral porphyrias: what a hematologist needs to know.
New type of acute porphyria with porphobilinogen synthase (delta-aminolevulinic acid dehydratase) defect in the homozygous state.
Novel molecular defects of the delta-aminolevulinate dehydratase gene in a patient with inherited acute hepatic porphyria.
Oxidative stress in mice is dependent on the free glucose content of the diet.
Pathogenesis and clinical features of the acute hepatic porphyrias (AHPs).
Porphobilinogen synthase: An equilibrium of different assemblies in human health.
Porphyria syndrome associated with diabetic nephrosclerosis and erythropoietin.
Porphyrin synthesis and mitochondrial respiration in acute intermittent porphyria: studies using cultured human fibroblasts.
Study of erythrocyte delta-aminolevulinic acid dehydratase activity in porphyria cutanea tarda.
The cutaneous porphyrias.
The third case of Doss porphyria (delta-amino-levulinic acid dehydratase deficiency) in Germany.
Treatment of the porphyrias.
Treatment options in acute porphyria, porphyria cutanea tarda, and erythropoietic protoporphyria.
[Drugs and porphyric crisis]
[Effect of ecological factors on the incidence of toxic porphyria. I. Alcoholism and delta-aminolevulinic acid dehydratase activity]
[Enzymatic studies on porphyria. II. Liver delta-aminolevulinic acid dehydrase activity in griseofulvin induced protoporphyria mice]
[Influence of ecologic factors on the phenomenon of toxic porphyria. II. Influence of alcohol on ALA-D activity in lead poisoning]
[Porphyrin-synthesizing enzymes in erythrocyte]
[Studies on the activity of 5-aminolevulinic acid dehydrase in the blood in experimental porphyria and in patients.]
[Treatment of acute porphyrias. The importance of follow-up of patients and carriers]
Porphyrias, Hepatic
"Glucose effect" and rate limiting function of uroporphyrinogen synthase on porphyrin metabolism in hepatocyte culture: relationship with human acute hepatic porphyrias.
Acute hepatic porphyria syndrome with porphobilinogen synthase defect.
Acute porphyrias: pathogenesis of neurological manifestations.
An acquired acute hepatic porphyria: a novel type of delta-aminolevulinate dehydratase inhibition.
Composition of urinary coproporphyrin isomers I-IV in human porphyrias.
delta-Aminolevulinic acid dehydrase (porphobilinogen synthase) in two families with inherited enzyme deficiency.
Enzymatic defect in a child with hereditary hepatic porphyria due to homozygous delta-aminolevulinic acid dehydratase deficiency: immunochemical studies.
Excretion pattern of faecal coproporphyrin isomers I-IV in human porphyrias.
Heme biosynthesis and the porphyrias.
Hereditary hepatic porphyria due to homozygous delta-aminolevulinic acid dehydratase deficiency: studies in lymphocytes and erythrocytes.
Hereditary hepatic porphyria with delta aminolevulinate dehydrase deficiency: immunologic characterization of the non-catalytic enzyme.
Human hereditary hepatic porphyrias.
Message amplification phenotyping of an inherited delta-aminolevulinate dehydratase deficiency in a family with acute hepatic porphyria.
New type of acute porphyria with porphobilinogen synthase (delta-aminolevulinic acid dehydratase) defect in the homozygous state.
New type of hepatic porphyria with porphobilinogen synthase defect and intermittent acute clinical manifestation.
Novel molecular defects of the delta-aminolevulinate dehydratase gene in a patient with inherited acute hepatic porphyria.
Pathogenesis and clinical features of the acute hepatic porphyrias (AHPs).
The "glucose effect" in acute hepatic porphyrias and in experimental porphyria.
Toxic effects of griseofulvin: disease models, mechanisms, and risk assessment.
Pre-Eclampsia
Delta-aminolevulinate dehydratase activity and oxidative stress markers in preeclampsia.
Protein-Energy Malnutrition
Influence of protein calorie malnutrition and fasting on the activities of delta-aminolevulinic acid dehydratase and porphobilinogen deaminase in rats.
Proteinuria
Role of selenium in protection against lead intoxication.
Protoporphyria, Erythropoietic
In inherited porphyrias, lead intoxication is a toxogenetic disorder.
Protoporphyrinaemia and decreased activities of 5-aminolevulinic acid dehydrase and uroporphyrinogen I synthetase in erythrocytes of a Vitamin B6-deficient epileptic boy given valproic acid and carbamazepine.
pyruvate kinase deficiency
Heme synthesis in hereditary hemolytic anemias: decreased delta-aminolevulinic acid synthetase in hemoglobin Köln disease.
Radiculopathy
Accuracy of Bony Gutter Placement in Cervical Laminoplasty Assisted by 3-D Print Modeling, and Associations with Posterior Spinal Cord Shift and Radiculopathy.
Renal Insufficiency
Effect of aluminum on porphyrin metabolism in hemodialyzed patients.
Effect of lycopene on nephrotoxicity induced by mercuric chloride in rats.
Reduced aminolevulinate dehydrase activity in rats with functional renal failure induced by cyclosporin A.
Reduced aminolevulinic dehydrase activity with increased blood porphyrins in experimental chronic renal failure.
Renal Insufficiency, Chronic
Studies on the biosynthesis of porphyrins in erythrocytes after incubation with delta-aminolevulinic acid: an attempt to investigate the pathogenesis of nephrogenic anemia.
Seizures
Diphenyl diselenide-induced seizures in rat pups: possible interaction with glutamatergic system.
Involvement of oxidative stress in seizures induced by diphenyl diselenide in rat pups.
Lipoic Acid Alters delta-Aminolevulinic Dehydratase, Glutathione Peroxidase and Na+,K+-ATPase Activities and Glutathione-Reduced Levels in Rat Hippocampus After Pilocarpine-Induced Seizures.
Lipoic acid blocks seizures induced by pilocarpine via increases in delta-aminolevulinic dehydratase and Na+, K+-ATPase activity in rat brain.
Neuropharmacological effects of lipoic acid and ubiquinone on delta-aminolevulinic dehydratase, Na, K-ATPase, and Mg-ATPase activities in rat hippocampus after pilocarpine-induced seizures.
Tyrosinemias
Detection of succinylacetone and the use of its measurement in mass screening for hereditary tyrosinemia.
Hereditary tyrosinemia and the heme biosynthetic pathway. Profound inhibition of delta-aminolevulinic acid dehydratase activity by succinylacetone.
Iron mobilization by succinylacetone methyl ester in rats. A model study for hereditary tyrosinemia and porphyrias characterized by 5-aminolevulinic acid overload.
On the enzymic defects in hereditary tyrosinemia.
Perturbation of maleylacetoacetic acid metabolism in rats with dichloroacetic Acid-induced glutathione transferase zeta deficiency.
Pitfalls in the initial diagnosis of tyrosinemia: three case reports and a review of the literature.
Renal heme metabolism in hereditary tyrosinemia: use of succinylacetone in rat renal tubules.
Spectrophotometric microassay for delta-aminolevulinate dehydratase in dried-blood spots as confirmation for hereditary tyrosinemia type I.
Succinylacetone and delta-aminolevulinic acid dehydratase in hereditary tyrosinemia: immunochemical study of the enzyme.
Succinylacetone inhibits delta-aminolevulinate dehydratase and potentiates the drug and steroid induction of delta-aminolevulinate synthase in liver.
Tyrosinemia with aminolevulinic dehydratase deficiency.
Uremia
Abnormalities of heme biosynthesis in experimental acute renal failure.
Oxidative stress and delta-ALA-D activity in chronic renal failure patients.
Urogenital Neoplasms
Case-only gene-environment interaction between ALAD tagSNPs and occupational lead exposure in prostate cancer.
Uterine Cervical Neoplasms
Involvement of oxidative stress in the pre-malignant and malignant states of cervical cancer in women.
Virus Diseases
Identification of intramural epithelial networks linked to peribiliary glands that express progenitor cell markers and proliferate after injury in mice.