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succinyl acetone
heptanoic acid, 4,6-dioxo-

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Name:4,6-dioxoheptanoic acid
CAS Number: 51568-18-4Picture of molecule3D/inchi
FDA UNII: Search
Nikkaji Web:J228.073F
XlogP3:-0.80 (est)
Molecular Weight:158.15350000
Formula:C7 H10 O4
NMR Predictor:Predict (works with chrome, Edge or firefox)
Category:information only not used for fragrances or flavors
US / EU / FDA / JECFA / FEMA / FLAVIS / Scholar / Patent Information:
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Physical Properties:
Assay: 95.00 to 100.00
Food Chemicals Codex Listed: No
Soluble in:
 water, 2.579e+005 mg/L @ 25 °C (est)
Organoleptic Properties:
Odor and/or flavor descriptions from others (if found).
Cosmetic Information:
None found
BOC Sciences
For experimental / research use only.
Odor: characteristic
Use: Succinylacetone is an inhibitor of -aminolevulinic acid dehydratase that inhi
Santa Cruz Biotechnology
For experimental / research use only.
Sigma-Aldrich: Sigma
For experimental / research use only.
4,6-Dioxoheptanoic acid powder
Safety Information:
Preferred SDS: View
Hazards identification
Classification of the substance or mixture
GHS Classification in accordance with 29 CFR 1910 (OSHA HCS)
None found.
GHS Label elements, including precautionary statements
Hazard statement(s)
None found.
Precautionary statement(s)
None found.
Oral/Parenteral Toxicity:
Not determined
Dermal Toxicity:
Not determined
Inhalation Toxicity:
Not determined
Safety in Use Information:
information only not used for fragrances or flavors
Recommendation for succinyl acetone usage levels up to:
 not for fragrance use.
Recommendation for succinyl acetone flavor usage levels up to:
 not for flavor use.
Safety References:
EPI System: View
Chemical Carcinogenesis Research Information System:Search
AIDS Citations:Search
Cancer Citations:Search
Toxicology Citations:Search
EPA ACToR:Toxicology Data
EPA Substance Registry Services (SRS):Registry
Laboratory Chemical Safety Summary :5312
National Institute of Allergy and Infectious Diseases:Data
WGK Germany:3
4,6-dioxoheptanoic acid
 4,6-dioxoheptanoic acid
NIST Chemistry WebBook:Search Inchi
Pubchem (cid):5312
Pubchem (sid):135057374
Other Information:
(IUPAC):Atomic Weights of the Elements 2011 (pdf)
Videos:The Periodic Table of Videos
tgsc:Atomic Weights use for this web site
(IUPAC):Periodic Table of the Elements
HMDB (The Human Metabolome Database):HMDB00635
Export Tariff Code:2918.30.5000
Potential Blenders and core components note
None Found
Potential Uses:
None Found
Occurrence (nature, food, other):note
 not found in nature
4,6-dioxoheptanoic acid
 heptanoic acid, 4,6-dioxo-


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.
inhibitor of heme biosynthesis. Succinylacetone is a tyrosine metabolite (PMID 16448836). It is a specific marker for Tyrosinemia type I. Type I tyrosinemia is an inherited metabolism disorder due to a shortage of the enzyme fumarylacetoacetate hydrolase that is needed to break down tyrosine. (Wikipedia) [HMDB]
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