| 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. |
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