succinylacetone has been researched along with Amino Acid Metabolism Disorders, Inborn in 41 studies
succinylacetone: inhibitor of heme biosynthesis
4,6-dioxoheptanoic acid : A dioxo monocarboxylic acid that is heptanoic acid in which oxo groups replace the hydrogens at positions 4 and 6. It is an abnormal metabolite of the tyrosine metabolic pathway and a marker for type 1 tyrosinaemia.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Results of NTBC therapy used in three infants with type I tyrosinemia who presented with acute liver failure are reported." | 3.70 | [Favorable outcome of treatment with NTBC of acute liver insufficiency disclosing hereditary tyrosinemia type I]. ( Barkaoui, E; Bernard, O; Debray, D; Habès, D; Ogier, H, 1999) |
| "Succinylacetone in urine was elevated in both, 30 and 79 mumol/mmol creatinine, respectively." | 1.28 | Hereditary tyrosinemia of chronic course without rickets and renal tubular dysfunction. ( Halvorsen, S; Kvittingen, EA; Steen-Johnsen, J; Søvik, O, 1990) |
| "Treating succinylacetone with hydroxylamine hydrochloride at a pH of less than 5 caused formation of a derivative separated by capillary gas chromatography into two isomers: 3-methyl-5- isoxazole propionate and 5-methyl-3- isoxazole propionate as their trimethylsilyl derivatives (molecular weight 227)." | 1.27 | Determination of urinary succinylacetone by capillary gas chromatography. ( Bowers, LD; Fregien, KD; Krivit, W; Ramnaraine, ML; Tuchman, M; Whitley, CB, 1984) |
| "Succinylacetone was measured in sera and urines from 19 patients with hereditary tyrosinemia." | 1.26 | Detection of succinylacetone and the use of its measurement in mass screening for hereditary tyrosinemia. ( Gagné, R; Grenier, A; Laberge, C; Lescault, A; Mamer, O, 1982) |
| "Succinylacetone was excreted in the urine from four patients, with hereditary tyrosinemia i." | 1.26 | Urinary excretion of succinylacetone and delta-aminolevulinic acid in patients with hereditary tyrosinemia. ( Baekmark, UB; Brandt, NJ; Christensen, E; Gregersen, N; Hjeds, H; Jacobsen, BB; Pedersen, JB, 1981) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 21 (51.22) | 18.7374 |
| 1990's | 20 (48.78) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| King, GS | 3 |
| MacKenzie, F | 2 |
| Pettit, BR | 4 |
| Leonard, JV | 4 |
| Steinmann, B | 2 |
| Gitzelmann, R | 1 |
| Kvittingen, EA | 7 |
| Stokke, O | 2 |
| Tuchman, M | 2 |
| Whitley, CB | 1 |
| Ramnaraine, ML | 2 |
| Bowers, LD | 1 |
| Fregien, KD | 1 |
| Krivit, W | 1 |
| Sassa, S | 4 |
| Kappas, A | 4 |
| Wadman, SK | 3 |
| Duran, M | 2 |
| Ketting, D | 2 |
| Bruinvis, L | 1 |
| van Sprang, FJ | 1 |
| Berger, R | 2 |
| Smit, GP | 3 |
| Divry, P | 1 |
| Farriaux, JP | 1 |
| Cartigny, B | 1 |
| Grenier, A | 3 |
| Lescault, A | 2 |
| Laberge, C | 3 |
| Gagné, R | 3 |
| Mamer, O | 1 |
| Lindblad, B | 2 |
| Steen, G | 2 |
| Fällström, SP | 1 |
| Stoker-de Vries, SA | 1 |
| Christensen, E | 1 |
| Jacobsen, BB | 1 |
| Gregersen, N | 1 |
| Hjeds, H | 1 |
| Pedersen, JB | 1 |
| Brandt, NJ | 1 |
| Baekmark, UB | 1 |
| Grompe, M | 1 |
| Lindstedt, S | 2 |
| al-Dhalimy, M | 1 |
| Kennaway, NG | 1 |
| Papaconstantinou, J | 1 |
| Torres-Ramos, CA | 1 |
| Ou, CN | 1 |
| Finegold, M | 1 |
| Pérez-Cerdá, C | 1 |
| Merinero, B | 1 |
| Sanz, P | 1 |
| Castro, M | 1 |
| Gangoiti, J | 1 |
| García, MJ | 1 |
| Díaz, M | 1 |
| Medina, E | 1 |
| Ugarte, M | 1 |
| Fernández-Cañón, JM | 1 |
| Peñalva, MA | 1 |
| Schierbeek, H | 1 |
| Beukeveld, GJ | 1 |
| van Faassen, H | 1 |
| van Spronsen, FJ | 1 |
| Bijsterveld, K | 1 |
| Venekamp-Hoolsema, EE | 1 |
| Wolthers, BG | 1 |
| Biasucci, G | 1 |
| Giuffré, B | 1 |
| La Grutta, S | 1 |
| Riva, E | 1 |
| Bruneau, N | 1 |
| St-Vil, D | 1 |
| Luks, FI | 1 |
| LaBerge, JM | 1 |
| Bensoussan, AL | 1 |
| Blanchard, H | 1 |
| Cederbaum, S | 1 |
| Jakobs, C | 3 |
| Tanguay, RM | 2 |
| Prieto-Alamo, MJ | 1 |
| Laval, F | 1 |
| Poudrier, J | 1 |
| Lettre, F | 1 |
| St-Louis, M | 1 |
| Barkaoui, E | 1 |
| Debray, D | 1 |
| Habès, D | 1 |
| Ogier, H | 1 |
| Bernard, O | 1 |
| Holme, E | 1 |
| Lock, EA | 1 |
| Hjalmarson, O | 1 |
| Strandvik, B | 1 |
| Valík, D | 1 |
| Klusácek, D | 1 |
| Pintera, J | 1 |
| Collins, JC | 1 |
| Buchanan, DN | 1 |
| Thoene, JG | 1 |
| Erickson, RP | 1 |
| Brooks, SS | 1 |
| Gluecksohn-Waelsch, S | 1 |
| Wyss, PA | 1 |
| Carter, BE | 2 |
| Boynton, SB | 1 |
| Connor, E | 1 |
| Fowler, B | 1 |
| Roth, KS | 3 |
| Rank, JM | 1 |
| Pascual-Leone, A | 1 |
| Payne, W | 1 |
| Glock, M | 1 |
| Freese, D | 1 |
| Sharp, H | 1 |
| Bloomer, JR | 2 |
| Søvik, O | 1 |
| Steen-Johnsen, J | 1 |
| Halvorsen, S | 2 |
| Moses, LC | 2 |
| Spencer, PD | 2 |
| Stellaard, F | 1 |
| Henderson, M | 1 |
| Lilford, R | 1 |
| Fujita, H | 1 |
| Bain, MD | 1 |
| Purkiss, P | 1 |
| Jones, M | 1 |
| Bingham, P | 1 |
| Stacey, TE | 1 |
| Chalmers, RA | 1 |
| Jellum, E | 1 |
| Flatmark, A | 1 |
| Bergan, A | 1 |
| Sødal, G | 1 |
| Schrumpf, E | 1 |
| Gjone, E | 1 |
| Medow, MS | 1 |
| Dorland, L | 1 |
| Wikkerink, B | 1 |
| Kok, RM | 1 |
| de Jong, AP | 1 |
| Brodtkorb, E | 1 |
| Freese, DK | 1 |
| Sharp, HL | 1 |
| Ascher, N | 1 |
| Manabe, S | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Pharmacotoxicology of Trichloroethylene Metabolites: Short-term Effect of DCA on in Vivo Tyrosine Catabolism and MAAI Expression[NCT00865514] | 2 participants (Actual) | Interventional | 2011-08-31 | Terminated (stopped due to Difficulty in obtaining the solution from the compounding pharmacies caused a two year delay in start-up; the funding ended prior to study completion.) | |||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
1 trial available for succinylacetone and Amino Acid Metabolism Disorders, Inborn
| Article | Year |
|---|---|
Hematin therapy for the neurologic crisis of tyrosinemia.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Aminolevulinic Acid; Heme; Hemin; Heptanoates; Hu | 1991 |
40 other studies available for succinylacetone and Amino Acid Metabolism Disorders, Inborn
| Article | Year |
|---|---|
Neonatal and prenatal diagnosis of hereditary tyrosinaemia.
Topics: Amino Acid Metabolism, Inborn Errors; Amniotic Fluid; Female; Heptanoates; Humans; Hydrolases; Infan | 1983 |
The antenatal diagnosis and aid to the management of hereditary tyrosinaemia by use of a specific and sensitive GC-MS assay for succinylacetone.
Topics: Amino Acid Metabolism, Inborn Errors; Amniotic Fluid; Female; Gas Chromatography-Mass Spectrometry; | 1984 |
Prenatal diagnosis of hereditary tyrosinemia.
Topics: Amino Acid Metabolism, Inborn Errors; Amniocentesis; Female; Heptanoates; Humans; Infant, Newborn; P | 1984 |
Determination of urinary succinylacetone by capillary gas chromatography.
Topics: Amino Acid Metabolism, Inborn Errors; Chromatography, Gas; Gas Chromatography-Mass Spectrometry; Hep | 1984 |
Hereditary tyrosinemia and the heme biosynthetic pathway. Profound inhibition of delta-aminolevulinic acid dehydratase activity by succinylacetone.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Cattle; Chick Embryo; Erythrocytes; Heme; Heptanoates | 1983 |
Urinary excretion of deuterated metabolites in patients with tyrosinemia type I after oral loading with deuterated L-tyrosine.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Acetoacetates; Amino Acid Metabolism, Inborn Errors; Deuterium; | 1983 |
Detection of succinylacetone and the use of its measurement in mass screening for hereditary tyrosinemia.
Topics: Amino Acid Metabolism, Inborn Errors; Heptanoates; Heptanoic Acids; Humans; Methods; Paper; Porphobi | 1982 |
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.
Topics: Acetoacetates; Amino Acid Metabolism, Inborn Errors; Gas Chromatography-Mass Spectrometry; Heptanoat | 1982 |
Succinylacetone inhibits delta-aminolevulinate dehydratase and potentiates the drug and steroid induction of delta-aminolevulinate synthase in liver.
Topics: 5-Aminolevulinate Synthetase; Amino Acid Metabolism, Inborn Errors; Animals; Chick Embryo; Erythrocy | 1982 |
On the renal tubular damage in hereditary tyrosinemia and on the formation of succinylacetoacetate and succinylacetone.
Topics: Acetoacetates; Amino Acid Metabolism, Inborn Errors; Aminolevulinic Acid; Child; Heptanoates; Homoge | 1981 |
Deficiency of fumarylacetoacetase in a patient with hereditary tyrosinemia.
Topics: Acetoacetates; Amino Acid Metabolism, Inborn Errors; Fumarates; Heptanoates; Humans; Hydrolases; Inf | 1981 |
["Succinylacetone effect' after oral homogentisate loading].
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Child; Erythrocytes; Female; Heptanoates; Heptanoic Aci | 1981 |
Urinary excretion of succinylacetone and delta-aminolevulinic acid in patients with hereditary tyrosinemia.
Topics: Acetoacetates; Amino Acid Metabolism, Inborn Errors; Aminolevulinic Acid; Female; Fumarates; Heptano | 1981 |
Pharmacological correction of neonatal lethal hepatic dysfunction in a murine model of hereditary tyrosinaemia type I.
Topics: alpha-Fetoproteins; Amino Acid Metabolism, Inborn Errors; Amino Acids; Animals; Cyclohexanones; Dise | 1995 |
Liver transplantation in nine Spanish patients with tyrosinaemia type I.
Topics: Amino Acid Metabolism, Inborn Errors; Aminolevulinic Acid; Enzyme Inhibitors; Graft Rejection; Hepta | 1995 |
Fungal metabolic model for human type I hereditary tyrosinaemia.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acid Sequence; Aspergillus nidulans; Chromatography, Hig | 1995 |
Hereditary tyrosinaemia type I: a long-term study of the relationship between the urinary excretions of succinylacetone and delta-aminolevulinic acid.
Topics: Amino Acid Metabolism, Inborn Errors; Aminolevulinic Acid; Child, Preschool; Creatinine; Heptanoates | 1993 |
Tyrosinaemia type Ia without excess of urinary succinylacetone.
Topics: Amino Acid Metabolism, Inborn Errors; Child, Preschool; Female; Heptanoates; Humans; Tyrosine | 1993 |
[Surgical and metabolic aspects of liver transplantation for tyrosinemia].
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Aminolevulinic Acid; Central Nervous System Diseas | 1993 |
A case of tyrosinaemia type I with normal level of succinylacetone in the amniotic fluid.
Topics: Amino Acid Metabolism, Inborn Errors; Amniotic Fluid; Cells, Cultured; DNA Mutational Analysis; Enzy | 1996 |
Deficient DNA-ligase activity in the metabolic disease tyrosinemia type I.
Topics: Amino Acid Metabolism, Inborn Errors; Cells, Cultured; DNA; DNA Ligases; Fibroblasts; Heptanoates; H | 1998 |
Genotyping of a case of tyrosinaemia type I with normal level of succinylacetone in amniotic fluid.
Topics: Amino Acid Metabolism, Inborn Errors; Amniotic Fluid; Female; Genotype; Heptanoates; Heterozygote; H | 1999 |
[Favorable outcome of treatment with NTBC of acute liver insufficiency disclosing hereditary tyrosinemia type I].
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Acute Disease; Amino Acid Metabolism, Inborn Errors; Aminolevul | 1999 |
Treatment of hereditary tyrosinaemia type I by inhibition of 4-hydroxyphenylpyruvate dioxygenase.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Acetoacetates; alpha-Fetoproteins; Amino Acid Metabolism, Inbor | 1992 |
Impaired synthesis of taurine in a patient with tyrosinemia type I during the oral L-methionine loading test.
Topics: Administration, Oral; Amino Acid Metabolism, Inborn Errors; Female; Gas Chromatography-Mass Spectrom | 1992 |
Metabolic studies in a mouse model of hepatorenal tyrosinemia: absence of perinatal abnormalities.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Animals, Newborn; Chromosome Deletion; Disease Models | 1992 |
Renal heme metabolism in hereditary tyrosinemia: use of succinylacetone in rat renal tubules.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Cytochrome P-450 Enzyme System; Cytosol; Ferrochelata | 1991 |
Hereditary tyrosinemia of chronic course without rickets and renal tubular dysfunction.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Amino Acids; Child; Child, Preschool; Chronic Kidn | 1990 |
On rat renal aminolevulinate transport and metabolism in experimental Fanconi syndrome.
Topics: Amino Acid Metabolism, Inborn Errors; Aminolevulinic Acid; Animals; Biological Transport, Active; Fa | 1990 |
First-trimester prenatal diagnosis of tyrosinemia type I by amniotic fluid succinylacetone determination.
Topics: Amino Acid Metabolism, Inborn Errors; Amniotic Fluid; Female; Heptanoates; Heptanoic Acids; Humans; | 1990 |
Succinylacetone and delta-aminolevulinic acid dehydratase in hereditary tyrosinemia: immunochemical study of the enzyme.
Topics: Amino Acid Metabolism, Inborn Errors; Child, Preschool; Electrophoresis, Polyacrylamide Gel; Erythro | 1990 |
Dietary treatment eliminates succinylacetone from the urine of a patient with tyrosinaemia type 1.
Topics: Amino Acid Metabolism, Inborn Errors; Female; Heme; Heptanoates; Heptanoic Acids; Humans; Infant; Me | 1990 |
Early prenatal diagnosis of hereditary tyrosinaemia.
Topics: Amino Acid Metabolism, Inborn Errors; Amniotic Fluid; Female; Heptanoates; Humans; Pregnancy; Pregna | 1985 |
Liver transplantation in a 23-year-old tyrosinaemia patient: effects on the renal tubular dysfunction.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids; beta 2-Microglobulin; Female; Glycosuria; | 1986 |
Effects of succinylacetone on the uptake of sugars and amino acids by brush border vesicles.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Animals; Biological Transport; Carbohydrate Metab | 1988 |
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.
Topics: Amino Acid Metabolism, Inborn Errors; Electrons; Female; Gas Chromatography-Mass Spectrometry; Hepta | 1988 |
The pre- and post-natal diagnosis of tyrosinemia type I and the detection of the carrier state by assay of fumarylacetoacetase.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Child; Female; Genetic Carrier Screening; Genetic Varia | 1986 |
Contribution of extrahepatic tissues to biochemical abnormalities in hereditary tyrosinemia type I: study of three patients after liver transplantation.
Topics: Acetoacetates; Amino Acid Metabolism, Inborn Errors; Aminolevulinic Acid; Child, Preschool; Female; | 1987 |
Hereditary tyrosinemia. Formation of succinylacetone-amino acid adducts.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Chromatography, High Pressure Liquid; Chromatogra | 1985 |
Concentrations of succinylacetone after homogentisate and tyrosine loading in healthy individuals with low fumarylacetoacetase activity.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Erythrocytes; Female; Fibroblasts; Gas Chromatography-M | 1985 |