succinyl acetone, 51568-18-4

Articles:

PubMed:	Clinical and Biochemical Profile of Tyrosinemia Type 1 in Tunisia.
PubMed:	Heme-mediated inhibition of Bach1 regulates the liver specificity and transience of the Nrf2-dependent induction of zebrafish heme oxygenase 1.
PubMed:	High-resolution metabolomics to discover potential parasite-specific biomarkers in a Plasmodium falciparum erythrocytic stage culture system.
PubMed:	Geographical and Ethnic Distribution of Mutations of the Fumarylacetoacetate Hydrolase Gene in Hereditary Tyrosinemia Type 1.
PubMed:	Diagnosis and treatment of hereditary tyrosinemia in Japan.
PubMed:	Infants with Tyrosinemia Type 1: Should phenylalanine be supplemented?
PubMed:	Two novel FAH gene mutations in a patient with hereditary tyrosinemia type I.
PubMed:	Inborn Error of Metabolism (IEM) screening in Singapore by electrospray ionization-tandem mass spectrometry (ESI/MS/MS): An 8 year journey from pilot to current program.
PubMed:	Cross-sectional study of 168 patients with hepatorenal tyrosinaemia and implications for clinical practice.
PubMed:	Succinylacetone as primary marker to detect tyrosinemia type I in newborns and its measurement by newborn screening programs.
PubMed:	Tyrosinemia type 1: an overview of nursing care.
PubMed:	Tyrosinemia type I: clinical and biochemical analysis of patients in Mexico.
PubMed:	Clinical and para clinical findings in the children with tyrosinemia referring for liver transplantation.
PubMed:	The inclusion of ADA-SCID in expanded newborn screening by tandem mass spectrometry.
PubMed:	Tyrosinemia type 1: a rare and forgotten cause of reversible hypertrophic cardiomyopathy in infancy.
PubMed:	[Mutation analysis of FAH gene in patients with tyrosinemia type 1].
PubMed:	Functional analysis and in vitro correction of splicing FAH mutations causing tyrosinemia type I.
PubMed:	Heme oxygenase-1 regulates matrix metalloproteinase MMP-1 secretion and chondrocyte cell death via Nox4 NADPH oxidase activity in chondrocytes.
PubMed:	Disruption of fumarylacetoacetate hydrolase causes spontaneous cell death under short-day conditions in Arabidopsis.
PubMed:	Heme status affects human hepatic messenger RNA and microRNA expression.
PubMed:	Newborn screening for hepatorenal tyrosinemia-I by tandem mass spectrometry using pooled samples: a four-year summary by the New England newborn screening program.
PubMed:	Tyrosinemia: a report of three cases from India.
PubMed:	Maternal and fetal tyrosinemia type I.
PubMed:	[Analysis of clinical data and genetic mutations in three Chinese patients with tyrosinemia type I].
PubMed:	Fumarylacetoacetate inhibits the initial step of the base excision repair pathway: implication for the pathogenesis of tyrosinemia type I.
PubMed:	Biochemical and molecular diagnosis of tyrosinemia type I with two novel FAH mutations in a Hong Kong chinese patient: recommendation for expanded newborn screening in Hong Kong.
PubMed:	Performance of succinylacetone assays and their associated proficiency testing outcomes.
PubMed:	Effect of nitisinone (NTBC) treatment on the clinical course of hepatorenal tyrosinemia in Québec.
PubMed:	Heme and heme biosynthesis intermediates induce heme oxygenase-1 and cytochrome P450 2A5, enzymes with putative sequential roles in heme and bilirubin metabolism: different requirement for transcription factor nuclear factor erythroid- derived 2-like 2.
PubMed:	Hepatorenal tyrosinemia.
PubMed:	Evaluation of factors influencing accuracy in the analysis of succinylacetone in dried blood spots.
PubMed:	Evaluation of a novel, commercially available mass spectrometry kit for newborn screening including succinylacetone without hydrazine.
PubMed:	Plasma succinylacetone is persistently raised after liver transplantation in tyrosinaemia type 1.
PubMed:	[Application of succinylacetone levels measurement in the blood and urine in the diagnosis of tyrosinemia type 1].
PubMed:	Dried blood spot analysis by digital microfluidics coupled to nanoelectrospray ionization mass spectrometry.
PubMed:	Single dose NTBC-treatment of hereditary tyrosinemia type I.
PubMed:	Impaired DNA repair and genomic stability in hereditary tyrosinemia type 1.
PubMed:	LC-MS/MS method for simultaneous determination on a dried blood spot of multiple analytes relevant for treatment monitoring in patients with tyrosinemia type I.
PubMed:	Long-term outcome of living donor liver transplantation in a Thai boy with hereditary tyrosinemia type I: a case report.
PubMed:	The stability of markers in dried-blood spots for recommended newborn screening disorders in the United States.
PubMed:	Functional significance of the ATP-binding cassette transporter B6 in hepatocellular carcinoma.
PubMed:	Tyrosinemia type 1 in Spain: mutational analysis, treatment and long-term outcome.
PubMed:	Hereditary tyrosinemia type 1 from a single center in Egypt: clinical study of 22 cases.
PubMed:	Newborn screening for Tyr-I: two years' experience of the New York State program.
PubMed:	Improved tandem mass spectrometry (MS/MS) derivatized method for the detection of tyrosinemia type I, amino acids and acylcarnitine disorders using a single extraction process.
PubMed:	Newborn Screening for Tyrosinemia Type I: Further Evidence that Succinylacetone Determination on Blood Spot Is Essential.
PubMed:	[Clinical, biochemical and molecular characteristics in 11 Czech children with tyrosinemia type I].
PubMed:	The role of heme and the mitochondrion in the chemical and molecular mechanisms of mammalian cell death induced by the artemisinin antimalarials.
PubMed:	Monitoring tyrosinaemia type I: Blood spot test for nitisinone (NTBC).
PubMed:	Hereditary tyrosinemia type 1 metabolites impair DNA excision repair pathways.
PubMed:	Plastid-associated porphobilinogen synthase from Toxoplasma gondii: kinetic and structural properties validate therapeutic potential.
PubMed:	Assessing the DNA methylation status of single cells with the comet assay.
PubMed:	Investigation of mechanism(s) of DNA damage induced by 4-monochlorobiphenyl (PCB3) metabolites.
PubMed:	Lack of hemodynamic effects after extended heme synthesis inhibition by succinylacetone in rats.
PubMed:	A novel mutation causing mild, atypical fumarylacetoacetase deficiency (Tyrosinemia type I): a case report.
PubMed:	The successful inclusion of succinylacetone as a marker of tyrosinemia type I in Tuscany newborn screening program.
PubMed:	Clinical practice. NTBC therapy for tyrosinemia type 1: how much is enough?
PubMed:	Preliminary proficiency testing results for succinylacetone in dried blood spots for newborn screening for tyrosinemia type I.
PubMed:	Solution structure of succinylacetone, an unsymmetrical beta-diketone, as studied by 13C NMR and GIAO-DFT calculations.
PubMed:	Identification of a neonate with hepatorenal tyrosinemia by combined routine newborn screening for succinylacetone, acylcarnitines and amino acids.
PubMed:	Significant increase of succinylacetone within the first 12 h of life in hereditary tyrosinemia type 1.
PubMed:	Clinical, biochemical, and genetic analysis of a Korean neonate with hereditary tyrosinemia type 1.
PubMed:	Improved MS/MS analysis of succinylacetone extracted from dried blood spots when combined with amino acids and acylcarnitine butyl esters.
PubMed:	Post-transcriptional regulation of the expression of ferrochelatase by its variant mRNA.
PubMed:	Progress in expanded newborn screening for metabolic conditions by LC-MS/MS in Tuscany: update on methods to reduce false tests.
PubMed:	Acute depletion of heme by succinylacetone alters vascular responses but does not induce hypertension.
PubMed:	Improved method to determine succinylacetone in dried blood spots for diagnosis of tyrosinemia type 1 using UPLC-MS/MS.
PubMed:	The effect of haem biosynthesis inhibitors and inducers on intestinal iron absorption and liver haem biosynthetic enzyme activities.
PubMed:	Enhancing newborn screening for tyrosinemia type I.
PubMed:	Combined newborn screening for succinylacetone, amino acids, and acylcarnitines in dried blood spots.
PubMed:	The inclusion of succinylacetone as marker for tyrosinemia type I in expanded newborn screening programs.
PubMed:	Hemin reduces cellular sensitivity to imatinib and anthracyclins via Nrf2.
PubMed:	Human tryptophanyl-tRNA synthetase binds with heme to enhance its aminoacylation activity.
PubMed:	Myeloperoxidase is a key regulator of oxidative stress mediated apoptosis in myeloid leukemic cells.
PubMed:	Tandem mass spectrometric determination of succinylacetone in dried blood spots enables presymptomatic detection in a case of hepatorenal tyrosinaemia.
PubMed:	Heme induces ubiquitination and degradation of the transcription factor Bach1.
PubMed:	A modified Girard derivatizing reagent for universal profiling and trace analysis of aldehydes and ketones by electrospray ionization tandem mass spectrometry.
PubMed:	Animal models of tyrosinemia.
PubMed:	Messenger RNA as a source of transposase for sleeping beauty transposon-mediated correction of hereditary tyrosinemia type I.
PubMed:	Porphyria syndrome associated with diabetic nephrosclerosis and erythropoietin.
PubMed:	Porphobilinogen synthase from the butterfly, Pieris brassicae: purification and comparative characterization.
PubMed:	Analysis of succinylacetone, as a Girard T derivative, in urine and dried bloodspots by flow injection electrospray ionization tandem mass spectrometry.
PubMed:	Slow-onset inhibition of fumarylacetoacetate hydrolase by phosphinate mimics of the tetrahedral intermediate: kinetics, crystal structure and pharmacokinetics.
PubMed:	Gene expression profiles of homogentisate-treated Fah-/- Hpd-/-mice using DNA microarrays.
PubMed:	Myeloperoxidase-catalyzed metabolism of etoposide to its quinone and glutathione adduct forms in HL60 cells.
PubMed:	Increased mutant frequencies in the HPRT gene locus of leukemia HL-60 cells treated with succinylacetone.
PubMed:	A GC-MS/MS method for the quantitative analysis of low levels of the tyrosine metabolites maleylacetone, succinylacetone, and the tyrosine metabolism inhibitor dichloroacetate in biological fluids and tissues.
PubMed:	5-Aminolevulinate and 4, 5-dioxovalerate ions decrease GABA(A) receptor density in neuronal cells, synaptosomes and rat brain.
PubMed:	The genetic tyrosinemias.
PubMed:	Tyrosinemia type I--diagnostic issues and prenatal diagnosis.
PubMed:	Sodium nitroprusside promotes IRP2 degradation via an increase in intracellular iron and in the absence of S nitrosylation at C178.
PubMed:	Quantitative determination of succinylacetone in dried blood spots for newborn screening of tyrosinemia type I.
PubMed:	Newborn screening for hepatorenal tyrosinemia: Tandem mass spectrometric quantification of succinylacetone.
PubMed:	Renal function in tyrosinaemia type I after liver transplantation: a long-term follow-up.
PubMed:	Remodeling the regulation of iron metabolism during erythroid differentiation to ensure efficient heme biosynthesis.
PubMed:	A GC/MS validated method for the nanomolar range determination of succinylacetone in amniotic fluid and plasma: an analytical tool for tyrosinemia type I.
PubMed:	Determination of succinylacetone in dried blood spots and liquid urine as a dansylhydrazone by liquid chromatography tandem mass spectrometry.
PubMed:	Involvement of endoplasmic reticulum stress in hereditary tyrosinemia type I.
PubMed:	Deficiency of glutathione transferase zeta causes oxidative stress and activation of antioxidant response pathways.
PubMed:	Quantification of succinylacetone in urine of hepatorenal tyrosinemia patients by HPLC with fluorescence detection.
PubMed:	Stability of 5-aminolevulinic acid on dried urine filter paper for a diagnostic marker of tyrosinemia type I.
PubMed:	Haem repression of the housekeeping 5-aminolaevulinic acid synthase gene in the hepatoma cell line LMH.
PubMed:	Heme deficiency suppresses the expression of key neuronal genes and causes neuronal cell death.
PubMed:	Tandem mass spectrometric assay of succinylacetone in urine for the diagnosis of hepatorenal tyrosinemia.
PubMed:	Determination of urinary succinylacetone by capillary electrophoresis for the diagnosis of tyrosinemia type I.
PubMed:	Tyrosinemia type I: a clinico-laboratory case report.
PubMed:	Newborn screening for hepatorenal tyrosinemia by tandem mass spectrometry: analysis of succinylacetone extracted from dried blood spots.
PubMed:	Heme-dependent up-regulation of the alpha-globin gene expression by transcriptional repressor Bach1 in erythroid cells.
PubMed:	Intracellular kinetics of iron in reticulocytes: evidence for endosome involvement in iron targeting to mitochondria.
PubMed:	Mice deficient in glutathione transferase zeta/maleylacetoacetate isomerase exhibit a range of pathological changes and elevated expression of alpha, mu, and pi class glutathione transferases.
PubMed:	Unified gas chromatographic-mass spectrometric method for quantitating tyrosine metabolites in urine and plasma.
PubMed:	Heme deficiency causes apoptosis but does not increase ROS generation in HeLa cells.
PubMed:	Succinylacetone oxidation by oxygen/peroxynitrite: a possible source of reactive intermediates in hereditary tyrosinemia type I.
PubMed:	Heme controls the expression of cell cycle regulators and cell growth in HeLa cells.
PubMed:	Glycine N -methyltransferase deficiency: a new patient with a novel mutation.
PubMed:	Heme positively regulates the expression of beta-globin at the locus control region via the transcriptional factor Bach1 in erythroid cells.
PubMed:	Delta-aminolevulinic acid dehydratase from Plasmodium falciparum: indigenous versus imported.
PubMed:	Pharmacologic or genetic ablation of maleylacetoacetate isomerase increases levels of toxic tyrosine catabolites in rodents.
PubMed:	Infectious and inflammatory stimuli decrease endothelial nitric oxide synthase activity in vitro.
PubMed:	The role of endogenous heme synthesis and degradation domain cysteines in cellular iron-dependent degradation of IRP2.
PubMed:	Perturbation of maleylacetoacetic acid metabolism in rats with dichloroacetic Acid-induced glutathione transferase zeta deficiency.
PubMed:	Synthesis and immunosuppressive activity of novel succinylacetone analogues.
PubMed:	CYP2E1 overexpression up-regulates both non-specific delta-aminolevulinate synthase and heme oxygenase-1 in the human hepatoma cell line HLE/2E1.
PubMed:	Mobilization of intracellular iron by analogs of pyridoxal isonicotinoyl hydrazone (PIH) is determined by the membrane permeability of the iron-chelator complexes.
PubMed:	Cytochrome P450 CYP1A1 accumulates in the cytosol of kidney and brain and is activated by heme.
PubMed:	Heme deficiency interferes with the Ras-mitogen-activated protein kinase signaling pathway and expression of a subset of neuronal genes.
PubMed:	Maleylacetoacetate isomerase (MAAI/GSTZ)-deficient mice reveal a glutathione-dependent nonenzymatic bypass in tyrosine catabolism.
PubMed:	An examination of heme action in gene expression: heme and heme deficiency affect the expression of diverse genes in erythroid k562 and neuronal PC12 cells.
PubMed:	Erythroid differentiation and protoporphyrin IX down-regulate frataxin expression in Friend cells: characterization of frataxin expression compared to molecules involved in iron metabolism and hemoglobinization.
PubMed:	Selective determination of mitochondrial chelatable iron in viable cells with a new fluorescent sensor.
PubMed:	Long-term therapy with NTBC and tyrosine-restricted diet in a murine model of hereditary tyrosinemia type I.
PubMed:	The x-ray structure of yeast 5-aminolaevulinic acid dehydratase complexed with substrate and three inhibitors.
PubMed:	Gas chromatographic-mass spectrometric screening for organic acidemias using dried urine filter paper: determination of alpha-ketoacids.
PubMed:	[Liver failure with coagulopathy in an infant with tyrosinemia].
PubMed:	Spectrophotometric microassay for delta-aminolevulinate dehydratase in dried-blood spots as confirmation for hereditary tyrosinemia type I.
PubMed:	Development of potential iron chelators for the treatment of Friedreich's ataxia: ligands that mobilize mitochondrial iron.
PubMed:	Multiple regulatory steps in erythroid heme biosynthesis.
PubMed:	Regulation of heme metabolism in rat hepatocytes and hepatocyte cell lines: delta-aminolevulinic acid synthase and heme oxygenase are regulated by different heme-dependent mechanisms.
PubMed:	Characterization of a human plasma membrane heme transporter in intestinal and hepatocyte cell lines.
PubMed:	Antioxidants inhibit indoleamine 2,3-dioxygenase in IFN-gamma-activated human macrophages: posttranslational regulation by pyrrolidine dithiocarbamate.
PubMed:	Heme biosynthesis and oogenesis in the blood-sucking bug, Rhodnius prolixus.
PubMed:	Hepatocellular carcinoma despite long-term survival in chronic tyrosinaemia I.
PubMed:	Point mutations in the murine fumarylacetoacetate hydrolase gene: Animal models for the human genetic disorder hereditary tyrosinemia type 1.
PubMed:	Cutaneous porphyria in a neonate with tyrosinaemia type 1.
PubMed:	Porphobilinogen synthase from pea: expression from an artificial gene, kinetic characterization, and novel implications for subunit interactions.
PubMed:	Myeloperoxidase-catalyzed phenoxyl radicals of vitamin E homologue, 2,2,5,7,8-pentamethyl- 6-hydroxychromane, do not induce oxidative stress in live HL-60 cells.
PubMed:	Iron mobilization by succinylacetone methyl ester in rats. A model study for hereditary tyrosinemia and porphyrias characterized by 5-aminolevulinic acid overload.
PubMed:	Erythropoietin induction in Hep3B cells is not affected by inhibition of heme biosynthesis.
PubMed:	A mouse model of renal tubular injury of tyrosinemia type 1: development of de Toni Fanconi syndrome and apoptosis of renal tubular cells in Fah/Hpd double mutant mice.
PubMed:	Indications and outcome of liver transplantation in tyrosinaemia type 1.
PubMed:	Cyclin B-dependent kinase and caspase-1 activation precedes mitochondrial dysfunction in fumarylacetoacetate-induced apoptosis.
PubMed:	Myeloperoxidase-catalyzed redox-cycling of phenol promotes lipid peroxidation and thiol oxidation in HL-60 cells.
PubMed:	Heme biosynthesis in a chicken hepatoma cell line (LMH): comparison with primary chick embryo liver cells (CELC).
PubMed:	Tissue variant effects of heme inhibitors on the mouse cytochrome c oxidase gene expression and catalytic activity of the enzyme complex.
PubMed:	Synthesis and biological evaluation of a pyoverdin-beta-lactam conjugate: a new type of arginine-specific cross-linking in aqueous solution.
PubMed:	A GC/MS/MS screening method for multiple organic acidemias from urine specimens.
PubMed:	Photodynamically induced effects in colon carcinoma cells (WiDr) by endogenous photosensitizers generated by incubation with 5-aminolaevulinic acid.
PubMed:	[Favorable outcome of treatment with NTBC of acute liver insufficiency disclosing hereditary tyrosinemia type I].
PubMed:	Bcl-XL induction during terminal differentiation of friend erythroleukaemia cells correlates with delay of apoptosis and loss of proliferative capacity but not with haemoglobinization.
PubMed:	Evidence for and consequences of chronic heme deficiency in Belgrade rat reticulocytes.
PubMed:	Genotyping of a case of tyrosinaemia type I with normal level of succinylacetone in amniotic fluid.
PubMed:	Deficient DNA-ligase activity in the metabolic disease tyrosinemia type I.
PubMed:	Tyrosinaemia type I and NTBC (2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione).
PubMed:	Tyrosinemia type I-like disease: a possible manifestation of 3-oxo-delta 4-steroid 5 beta-reductase deficiency.
PubMed:	Heme synthesizing enzymes of Plasmodium knowlesi: a simian malaria parasite.
PubMed:	Hypoxic regulation of endothelial glyceraldehyde-3-phosphate dehydrogenase.
PubMed:	Characterization of a fungal maleylacetoacetate isomerase gene and identification of its human homologue.
PubMed:	Haem precursor delta-aminolaevulinic acid induces activation of the cytosolic iron regulatory protein 1.
PubMed:	Biosynthesis of the phagocyte NADPH oxidase cytochrome b558. Role of heme incorporation and heterodimer formation in maturation and stability of gp91phox and p22phox subunits.
PubMed:	Complete rescue of lethal albino c14CoS mice by null mutation of 4-hydroxyphenylpyruvate dioxygenase and induction of apoptosis of hepatocytes in these mice by in vivo retrieval of the tyrosine catabolic pathway.
PubMed:	Utilization of intracellular ferritin iron for hemoglobin synthesis in developing human erythroid precursors.
PubMed:	Characterization of a recombinant pea 5-aminolevulinic acid dehydratase and comparative inhibition studies with the Escherichia coli dehydratase.
PubMed:	Importance of haem biosynthesis in the control of intestinal iron absorption.
PubMed:	Comparative studies on the 5-aminolaevulinic acid dehydratases from Pisum sativum, Escherichia coli and Saccharomyces cerevisiae.
PubMed:	Imaging features of type 1 hereditary tyrosinemia: a review of 30 patients.
PubMed:	Tyrosinemia: the Quebec experience.
PubMed:	Hepatic 5-aminolevulinic acid synthase mRNA stability is modulated by inhibitors of heme biosynthesis and by metalloporphyrins.
PubMed:	Inhibition of renal uptake of indium-111-DTPA-octreotide in vivo.
PubMed:	Control of hemoglobin synthesis in erythroid differentiating K562 cells. I. Role of iron in erythroid cell heme synthesis.
PubMed:	Distribution of iron in reticulocytes after inhibition of heme synthesis with succinylacetone: examination of the intermediates involved in iron metabolism.
PubMed:	A case of tyrosinaemia type I with normal level of succinylacetone in the amniotic fluid.
PubMed:	Increased delta aminolevulinic acid and decreased pineal melatonin production. A common event in acute porphyria studies in the rat.
PubMed:	Fungal metabolic model for human type I hereditary tyrosinaemia.
PubMed:	Pharmacological correction of neonatal lethal hepatic dysfunction in a murine model of hereditary tyrosinaemia type I.
PubMed:	[delta-Aminolevulinate dehydratase deficiency].
PubMed:	Hemin-induced acceleration of hemoglobin production in immature cultured erythroid cells: preferential enhancement of fetal hemoglobin.
PubMed:	The nephropathy of type I tyrosinemia after liver transplantation.
PubMed:	Increased expression of opioid delta receptors by deoxy conformation heme proteins in NG108-15 cells.
PubMed:	Inhibition of heme synthesis induces apoptosis in human erythroid progenitor cells.
PubMed:	Liver transplantation in nine Spanish patients with tyrosinaemia type I.
PubMed:	Modulation of ornithine aminotransferase activity by oxygen in rat hepatocyte cultures.
PubMed:	Expression of a biologically active plant cytochrome b5 in Escherichia coli.
PubMed:	The post-translational processing of myeloperoxidase is regulated by the availability of heme.
PubMed:	Repression of ALA synthase by heme and zinc-mesoporphyrin in a chick embryo liver cell culture model of acute porphyria.
PubMed:	Regulation of transferrin receptor mRNA expression. Distinct regulatory features in erythroid cells.
PubMed:	Serum levels of oncofetal markers CA 125, CA 19-9, and alpha-fetoprotein in children with hereditary tyrosinemia type I.
PubMed:	Tissue distribution of succinylacetone in the rat in vivo: a possible basis for neurotoxicity in hereditary infantile tyrosinemia.
PubMed:	Peripheral neuropathy as the presenting feature of tyrosinaemia type I and effectively treated with an inhibitor of 4-hydroxyphenylpyruvate dioxygenase.
PubMed:	Iron distribution in Belgrade rat reticulocytes after inhibition of heme synthesis with succinylacetone.
PubMed:	Cloning and characterization of K562 cells on hemoglobin synthetic activity.
PubMed:	Ferrochelatase, glutathione peroxidase and transferrin receptor mRNA synthesis and levels in mouse erythroleukemia cells.
PubMed:	Clinical features and diagnostic approach in type I tyrosinaemia in an infant with cytomegaly virus infection and bacterial sepsis.
PubMed:	Dimethyl sulphoxide and haemin induce ferrochelatase mRNA by different mechanisms in murine erythroleukaemia cells.
PubMed:	Suppression of graft-versus-host disease by succinyl acetone in a rat allogeneic bone marrow transplantation model.
PubMed:	[Surgical and metabolic aspects of liver transplantation for tyrosinemia].
PubMed:	Tyrosinaemia type Ia without excess of urinary succinylacetone.
PubMed:	Hereditary tyrosinaemia type I: a long-term study of the relationship between the urinary excretions of succinylacetone and delta-aminolevulinic acid.
PubMed:	[Ferrochelatase gene expression in erythroid cells].
PubMed:	Roles of heme insertion and the mannose-6-phosphate receptor in processing of the human myeloid lysosomal enzyme, myeloperoxidase.
PubMed:	Inhibition of human lymphoproliferative responses and altered lymphocyte membrane phenotype by succinylacetone.
PubMed:	Succinyl acetone plus methotrexate as graft-versus-host disease prophylaxis in DLA-haploidentical canine littermate marrow grafts.
PubMed:	Pharmacologic, toxicologic, and marrow transplantation studies in dogs given succinyl acetone.
PubMed:	Modulation of the glucagon-dependent activation of the phosphoenolpyruvate carboxykinase gene by oxygen in rat hepatocyte cultures. Evidence for a heme protein as oxygen sensor.
PubMed:	Treatment of hereditary tyrosinaemia type I by inhibition of 4-hydroxyphenylpyruvate dioxygenase.
PubMed:	Impaired synthesis of taurine in a patient with tyrosinemia type I during the oral L-methionine loading test.
PubMed:	de novo biosynthesis of heme offers a new chemotherapeutic target in the human malarial parasite.
PubMed:	Metabolic studies in a mouse model of hepatorenal tyrosinemia: absence of perinatal abnormalities.
PubMed:	Ultrastructural, immunochemical, and cytochemical study of myeloperoxidase in myeloid leukemia HL-60 cells following treatment with succinylacetone, an inhibitor of heme biosynthesis.
PubMed:	delta-Aminolevulinic acid dehydratase: effects of succinylacetone in rat liver and kidney in an in vivo model of the renal Fanconi syndrome.
PubMed:	Physiological basis for an animal model of the renal Fanconi syndrome: use of succinylacetone in the rat.
PubMed:	Rapid improvement in the renal tubular dysfunction associated with tyrosinemia following hepatic replacement.
PubMed:	Inhibition of heme synthesis decreases transferrin receptor expression in mouse erythroleukemia cells.
PubMed:	Comparative effects of 3'-azido-3'-deoxythymidine and its metabolite 3'-amino-3'-deoxythymidine on hemoglobin synthesis in K-562 human leukemia cells.
PubMed:	Renal heme metabolism in hereditary tyrosinemia: use of succinylacetone in rat renal tubules.
PubMed:	Intracellular heme coordinately modulates globin chain synthesis, transferrin receptor number, and ferritin content in differentiating Friend erythroleukemia cells.
PubMed:	Involvement of heme in the transcriptional activation of CYPIIB1/B2 gene by phenobarbitone in rat liver--studies with succinylacetone.
PubMed:	Erythroleukemia differentiation. Distinctive responses of the erythroid-specific and the nonspecific delta-aminolevulinate synthase mRNA.
PubMed:	A murine model for type III tyrosinemia: lack of immunologically detectable 4-hydroxyphenylpyruvic acid dioxygenase enzyme protein in a novel mouse strain with hypertyrosinemia.
PubMed:	Urinary succinylacetone presence and delta-aminolaevulinic acid excretion in patients with type I tyrosinaemia during treatment.
PubMed:	Succinylacetone effects on renal tubular phosphate metabolism: a model for experimental renal Fanconi syndrome.
PubMed:	Regulation of ferritin and heme oxygenase synthesis in rat fibroblasts by different forms of iron.
PubMed:	Hematin therapy for the neurologic crisis of tyrosinemia.
PubMed:	Renal failure in adult patients with hereditary tyrosinaemia type I.
PubMed:	Tyrosinaemia type I--an update.
PubMed:	On rat renal aminolevulinate transport and metabolism in experimental Fanconi syndrome.
PubMed:	Hereditary tyrosinemia of chronic course without rickets and renal tubular dysfunction.
PubMed:	Role of heme in phenobarbital induction of cytochromes P450 and 5-aminolevulinate synthase in cultured rat hepatocytes maintained on an extracellular matrix.
PubMed:	Oral loading of homogentisic acid in controls and in obligate heterozygotes for hereditary tyrosinemia type I.
PubMed:	Fumarylacetoacetase measurement as a mass-screening procedure for hereditary tyrosinemia type I.
PubMed:	Succinylacetone and delta-aminolevulinic acid dehydratase in hereditary tyrosinemia: immunochemical study of the enzyme.
PubMed:	Dietary treatment eliminates succinylacetone from the urine of a patient with tyrosinaemia type 1.
PubMed:	Transferrin-polycation-mediated introduction of DNA into human leukemic cells: stimulation by agents that affect the survival of transfected DNA or modulate transferrin receptor levels.
PubMed:	An acute form of tyrosinemia type I with multiple intrahepatic mass lesions.
PubMed:	Mechanism of synergistic induction of hepatic heme oxygenase by glutethimide and iron: studies in cultured chick embryo liver cells.
PubMed:	Researchers follow varied molecular paths toward better control of organ rejection.
PubMed:	Neurologic crises in hereditary tyrosinemia.
PubMed:	First-trimester prenatal diagnosis of tyrosinemia type I by amniotic fluid succinylacetone determination.
PubMed:	Iron bound to low MW ligands: interactions with mitochondria and cytosolic proteins.
PubMed:	delta-Aminolevulinic acid dehydratase: is there a form unique to renal cortex?
PubMed:	Renal Fanconi syndrome: developmental basis for a new animal model with relevance to human disease.
PubMed:	Determination of succinylacetone and succinylacetoacetate in physiological samples as the common product 5(3)-methyl-3(5)-isoxazole propionic acid using an isotope dilution method and mass spectrometry.
PubMed:	delta-Aminolaevulinate synthase in human HepG2 hepatoma cells. Repression by haemin and induction by chemicals.
PubMed:	Erythropoietin controls heme metabolic enzymes in normal human bone marrow culture.
PubMed:	Evidence for an intercellular covalent reaction essential in antigen-specific T cell activation.
PubMed:	The effects of inhibition of heme synthesis on the intracellular localization of iron in rat reticulocytes.
PubMed:	Differentiating activity of adriamycin in human erythroleukemic cells: effect on globin and heme synthesis.
PubMed:	Effect of haem on cytochrome P-450 synthesis.
PubMed:	Heme may not be a positive regulator of cytochrome-P450 gene expression.
PubMed:	Human renal Fanconi syndrome--then and now.
PubMed:	Effects of succinylacetone on the uptake of sugars and amino acids by brush border vesicles.
PubMed:	Possible control of transcript levels by chlorophyll precursors in Chlamydomonas.
PubMed:	Increase in surface expression of transferrin receptors on cultured hepatocytes of adult rats in response to iron deficiency.
PubMed:	Treatment of experimental autoimmune uveitis in the rat with systemic succinylacetone.
PubMed:	Regulation of 5-aminolevulinate synthase in mouse erythroleukemic cells is different from that in liver.
PubMed:	[Sepsis, as the presenting form of hereditary tyrosinemia type I].
PubMed:	Haem inhibits iron uptake subsequent to endocytosis of transferrin in reticulocytes.
PubMed:	Xenobiotic mediated inhibition of hepatic uroporphyrinogen decarboxylase activity in 17-day-old chick embryo liver cells in culture.
PubMed:	Stable isotope dilution analysis of succinylacetone using electron capture negative ion mass fragmentography: an accurate approach to the pre- and neonatal diagnosis of hereditary tyrosinemia type I.
PubMed:	Routine gas chromatographic/mass spectrometric analysis of urinary organic acids. Results over a three-year period.
PubMed:	Immunosuppression by succinylacetone. II. Prevention of graft-vs-host disease.
PubMed:	Effect of succinylacetone administration on brain heme metabolism and behavior in mice.
PubMed:	Contribution of extrahepatic tissues to biochemical abnormalities in hereditary tyrosinemia type I: study of three patients after liver transplantation.
PubMed:	Effects of succinylacetone on amino acid uptake in the rat kidney.
PubMed:	Induction by hemin of proliferation and of differentiation of progenitor erythroid cells responsible for erythropoietin.
PubMed:	Porphobilinogen deaminase is unstable in the absence of its substrate.
PubMed:	Myoglobin expression in L6 muscle cells. Role of differentiation and heme.
PubMed:	Measurement of 5-aminolevulinic acid synthase activity in whole and fractionated human bone marrow: effect of myeloid cell lysis by monoclonal antibody.
PubMed:	The pre- and post-natal diagnosis of tyrosinemia type I and the detection of the carrier state by assay of fumarylacetoacetase.
PubMed:	Hereditary tyrosinemia type I--an overview.
PubMed:	Liver transplantation in a 23-year-old tyrosinaemia patient: effects on the renal tubular dysfunction.
PubMed:	Concentrations of succinylacetone after homogentisate and tyrosine loading in healthy individuals with low fumarylacetoacetase activity.
PubMed:	Effects of succinylacetone on methyl alpha-D-glucoside uptake by the rat renal tubule.
PubMed:	Heme requirement and acquisition by extracellular and intracellular stages of Leishmania mexicana amazonensis.
PubMed:	Hereditary tyrosinemia. Formation of succinylacetone-amino acid adducts.
PubMed:	Heme metabolism and erythropoiesis in abnormal iron states: role of delta-aminolevulinic acid synthase and heme oxygenase.
PubMed:	Accumulation of porphobilinogen deaminase, uroporphyrinogen decarboxylase, and alpha- and beta-globin mRNAs during differentiation of mouse erythroleukemic cells. Effects of succinylacetone.
PubMed:	Iron metabolism in K562 erythroleukemic cells.
PubMed:	Early prenatal diagnosis of hereditary tyrosinaemia.
PubMed:	Mobilization of ferritin iron in erythroblasts by chelating agents.
PubMed:	Effects of succinylacetone on growth and respiration of L1210 leukemia cells.
PubMed:	Regulation of heme synthesis in erythroid cells: hemin inhibits transferrin iron utilization but not protoporphyrin synthesis.
PubMed:	Augmentation of hematoporphyrin uptake and in vitro-growth inhibition of L1210 leukemia cells by succinylacetone.
PubMed:	Persistent succinylacetone excretion after liver transplantation in a patient with hereditary tyrosinaemia type I.
PubMed:	Biochemical studies of a patient with hereditary hepatorenal tyrosinemia: evidence of glutathione deficiency.
PubMed:	Heme regulation of HeLa cell transferrin receptor number.
PubMed:	Heme inhibits transferrin endocytosis in immature erythroid cells.
PubMed:	Effects of succinylacetone on dimethylsulfoxide-mediated induction of heme pathway enzymes in mouse friend virus-transformed erythroleukemia cells.
PubMed:	Induction of delta-aminolevulinic acid dehydratase in mouse Friend virus-transformed erythroleukemia cells during erythroid differentiation.
PubMed:	Persistent tyrosinemia associated with low activity of tyrosine aminotransferase.
PubMed:	Determination of urinary succinylacetone by capillary gas chromatography.
PubMed:	Chemical analysis of succinylacetone and 4-hydroxyphenyllactate in amniotic fluid using selective ion monitoring.
PubMed:	Prenatal diagnosis of hereditary tyrosinemia.
PubMed:	The antenatal diagnosis and aid to the management of hereditary tyrosinaemia by use of a specific and sensitive GC-MS assay for succinylacetone.
PubMed:	Biochemical studies on the enzymatic deficiencies in hereditary tyrosinemia.
PubMed:	The effect of N-methylprotoporphyrin and succinyl-acetone on the regulation of heme biosynthesis in chicken hepatocytes in culture.
PubMed:	Neonatal and prenatal diagnosis of hereditary tyrosinaemia.
PubMed:	Urinary excretion of deuterated metabolites in patients with tyrosinemia type I after oral loading with deuterated L-tyrosine.
PubMed:	Effect of succinylacetone on heme and cytochrome P450 synthesis in hepatocyte culture.
PubMed:	Hereditary tyrosinemia and the heme biosynthetic pathway. Profound inhibition of delta-aminolevulinic acid dehydratase activity by succinylacetone.
PubMed:	Growth inhibitory activity of succinylacetone: studies with Walker 256 carcinosarcoma, Novikoff hepatoma and L1210 leukemia.
PubMed:	Inhibition of heme synthesis in bone marrow cells by succinylacetone: effect on globin synthesis.
PubMed:	The effects of succinylacetone (4,6-dioxoheptanoic acid) on delta-aminolevulinate synthase activity and the content of heme in monolayers of chick embryo liver cells.
PubMed:	Neurotoxic aspects of porphyrinopathies: lead and succinylacetone.
PubMed:	Effects of altered porphyrin synthesis on brain neurochemistry.
PubMed:	Identification of 4,6-dioxoheptanoic acid (succinylacetone), 3,5-dioxooctanedioic acid (succinylacetoacetate) and 4-Oxo-6-hydroxyheptanoic acid in the urine from patients with hereditary tyrosinemia.
PubMed:	Detection of succinylacetone and the use of its measurement in mass screening for hereditary tyrosinemia.
PubMed:	Studied on cerebellar haem metabolism in the rat in vivo.
PubMed:	Prenatal diagnosis of hereditary tyrosinaemia: measurement of succinylacetone in amniotic fluid.
PubMed:	Immunosuppressive activity of succinylacetone.
PubMed:	Iron utilization in rabbit reticulocytes. A study using succinylacetone as an inhibitor or heme synthesis.
PubMed:	Succinylacetone inhibits delta-aminolevulinate dehydratase and potentiates the drug and steroid induction of delta-aminolevulinate synthase in liver.
PubMed:	Urinary excretion of succinylacetone and delta-aminolevulinic acid in patients with hereditary tyrosinemia.
PubMed:	Succinylacetone pyrrole, a powerful inhibitor of vitamin B12 biosynthesis: effect of delta-aminolevulinic acid dehydratase.
PubMed:	Inhibition of delta-aminolevulinic acid dehydrase by 4,6-dioxoheptanoic acid.
PubMed:	Deficiency of fumarylacetoacetase in a patient with hereditary tyrosinemia.
PubMed:	["Succinylacetone effect' after oral homogentisate loading].
PubMed:	The stimulation of globin synthesis by cobalt in reticulocytes with inhibited heme synthesis.
PubMed:	Uptake of hematin and growth of malignant murine erythroleukemia cells depleted of endogenous heme by succinylacetone.
PubMed:	On the renal tubular damage in hereditary tyrosinemia and on the formation of succinylacetoacetate and succinylacetone.
PubMed:	Succinylacetone, a potent inhibitor of heme biosynthesis: effect on cell growth, heme content and delta-aminolevulinic acid dehydratase activity of malignant murine erythroleukemia cells.
PubMed:	On the enzymic defects in hereditary tyrosinemia.