Page last updated: 2024-10-19

phenylpyruvic acid and BH4 Deficiency

phenylpyruvic acid has been researched along with BH4 Deficiency in 55 studies

phenylpyruvic acid: RN given refers to parent cpd
phenylpyruvate : A 2-oxo monocarboxylic acid anion resulting from deprotonation of the carboxy group of either keto- or enol-phenylpyruvic acid.
keto-phenylpyruvic acid : A 2-oxo monocarboxylic acid that is 3-phenylpropanoic acid substituted by an oxo group at position 2. It is an intermediate metabolite in the phenylalanine pathway.

Research Excerpts

ExcerptRelevanceReference
"Protein substitutes developed for phenylketonuria (PKU) are a synthetic source of protein commonly based on L-amino acids."2.72Protein Substitutes in PKU; Their Historical Evolution. ( Ashmore, C; Daly, A; Evans, S; MacDonald, A; Pinto, A, 2021)
"The inborn error of metabolism phenylketonuria (PKU, OMIM 261600) is most often due to inactivation of phenylalanine hydroxylase (PAH), which converts phenylalanine (Phe) into tyrosine (Tyr)."1.91Competitive, multi-objective, and compartmented Flux Balance Analysis for addressing tissue-specific inborn errors of metabolism. ( Liu, Y; Westerhoff, HV, 2023)
"Phenylketonuria is an autosomal recessive disorder caused by a deficiency of phenylalanine hydroxylase."1.36Tissue-specific activation of mitogen-activated protein kinases for expression of transthyretin by phenylalanine and its metabolite, phenylpyruvic acid. ( Choi, JO; Jung, SC; Lee, MH; Park, HY; Park, JW, 2010)

Research

Studies (55)

TimeframeStudies, this research(%)All Research%
pre-199040 (72.73)18.7374
1990's5 (9.09)18.2507
2000's5 (9.09)29.6817
2010's3 (5.45)24.3611
2020's2 (3.64)2.80

Authors

AuthorsStudies
Liu, Y1
Westerhoff, HV1
Daly, A1
Evans, S1
Pinto, A1
Ashmore, C1
MacDonald, A1
Sanayama, Y1
Matsumoto, A1
Shimojo, N1
Kohno, Y1
Nakaya, H1
MUNRO, TA1
Park, JW1
Lee, MH1
Choi, JO1
Park, HY1
Jung, SC1
PENROSE, LS2
Zhang, D1
Li, W1
Zhang, J1
Tang, W1
Qian, C1
Feng, M1
Chu, Q1
Ye, J1
Gazit, V1
Ben-Abraham, R1
Rudin, M1
Katz, Y1
Christ, SE1
JERVIS, GA1
ARMSTRONG, MD2
LOW, NL2
KNOX, WE2
HSIA, DY1
THE, TP1
FLEURY, P1
VINK, CL2
JONXIS, JH1
STRODER, J1
GEISLER, E1
VIZET, J1
SMITH, AJ1
STRANG, LB1
ASPERGER, H1
FOLLING, A1
SYDNES, S2
TASHIAN, RE1
WOOLF, LI1
BISAZ, S1
FARQUHAR, JW2
KANSAS, ET1
TAIT, HP2
HABERLANDT, WF1
HUMBEL, R1
SCHATZ, F1
RICHMOND, J1
CHRISTIAENS, L1
GAUDIER, B1
BRIET, B1
KRUPANIDHI, I1
PUNEKAR, BD1
GOSWAMI, ND1
LYNCH, RD1
DAVID, JM1
HASSAN, MM1
ALLEN, RJ1
WILSON, JL1
WOOLLEY, DW1
VANDERHOEVEN, T1
WHITEHEAD, RG1
MILBURN, TR1
ALLEGRANZA, A1
ALDEGHI, E1
CANEVINI, P1
DEVITO, C1
WU, WY1
SVENNEBY, G1
KVAMME, E1
EITINGER, L1
RUPE, CO1
FREE, AH1
COWIE, VA1
Langenbeck, U2
Behbehani, A2
Luthe, H1
Dorland, L1
Poll-The, BT1
Duran, M1
Smeitink, JA1
Berger, R1
Vilaseca, MA1
Farré, C1
Ramón, F1
Sarkissian, CN1
Scriver, CR1
Mamer, OA1
Fischer, GM1
Nemeti, B1
Farkas, V1
Debreceni, B1
Laszlo, A1
Schaffer, Z1
Somogyi, C1
Sandor, A1
Berti, SL1
Bonan, CD1
da Silva, FL1
Battastini, AM1
Sarkis, JJ1
Wannmacher, CM1
Mench-Hoinowski, A1
Kishida, M1
Makita, Y1
Suzuki, T1
Yamada, M1
Hobo, T1
Mönch, E1
Kneer, J1
Jakobs, C1
Arnold, M1
Diehl, H1
Batzler, U1
Antoshechkin, AG1
Zuyeva, LA1
Maximova, LA1
Hsiao, KJ1
Hung, SH1
Wu, SJ1
Yeh, SF1
Michals, K1
Matalon, R1

Reviews

2 reviews available for phenylpyruvic acid and BH4 Deficiency

ArticleYear
Protein Substitutes in PKU; Their Historical Evolution.
    Nutrients, 2021, Feb-02, Volume: 13, Issue:2

    Topics: Amino Acids; Animals; Caseins; Child, Preschool; Dietary Proteins; Female; History, 20th Century; Hi

2021
PHENYLKETONURIA IN PEDIATRIC PRACTICE.
    Clinical pediatrics, 1963, Volume: 2

    Topics: Child; Diet; Diet Therapy; Humans; Intelligence; Phenylalanine; Phenylketonurias; Phenylpyruvic Acid

1963

Other Studies

53 other studies available for phenylpyruvic acid and BH4 Deficiency

ArticleYear
Competitive, multi-objective, and compartmented Flux Balance Analysis for addressing tissue-specific inborn errors of metabolism.
    Journal of inherited metabolic disease, 2023, Volume: 46, Issue:4

    Topics: Humans; Phenylalanine; Phenylalanine Hydroxylase; Phenylketonurias; Phenylpyruvic Acids; Tyrosine

2023
Phenylalanine sensitive K562-D cells for the analysis of the biochemical impact of excess amino acid.
    Scientific reports, 2014, Nov-06, Volume: 4

    Topics: Adaptor Proteins, Signal Transducing; Biological Transport; Cell Differentiation; Cell Proliferation

2014
Phenylketonuria; data on 47 British families.
    Annals of eugenics, 1947, Volume: 14, Issue:pt 1

    Topics: Ethnicity; Humans; Phenylketonurias; Phenylpyruvic Acids; Urine

1947
Tissue-specific activation of mitogen-activated protein kinases for expression of transthyretin by phenylalanine and its metabolite, phenylpyruvic acid.
    Experimental & molecular medicine, 2010, Feb-28, Volume: 42, Issue:2

    Topics: Animals; Brain Stem; Disease Models, Animal; Gene Expression Regulation; Hep G2 Cells; Hepatocyte Nu

2010
Asearch for linkage between the A,B,C agglutinogens and phenylketonuria.
    American journal of mental deficiency, 1945, Volume: 50

    Topics: Antigens; Blood Group Antigens; Body Fluids; Genetic Linkage; Humans; Phenylketonurias; Phenylpyruvi

1945
Study on urinary metabolic profile of phenylketonuria by micellar electrokinetic capillary chromatography with dual electrochemical detection--potential clinical application in fast diagnosis of phenylketonuria.
    Analytica chimica acta, 2011, May-23, Volume: 694, Issue:1-2

    Topics: Chromatography, Micellar Electrokinetic Capillary; Electrochemical Techniques; Humans; Hydrogen-Ion

2011
Glucose-lowering effect of beta-phenylpyruvate in neonatal mice: a possible mechanism for phenylketonuria-related neurodegenerative changes.
    Brain research. Developmental brain research, 2003, Mar-14, Volume: 141, Issue:1-2

    Topics: Animals; Animals, Newborn; Blood Glucose; Brain; Disease Models, Animal; Dose-Response Relationship,

2003
Asbjørn Følling and the discovery of phenylketonuria.
    Journal of the history of the neurosciences, 2003, Volume: 12, Issue:1

    Topics: Female; History, 19th Century; History, 20th Century; Humans; Male; Norway; Phenylketonurias; Phenyl

2003
Studies on phenylpyruvic oligophrenia; phenylpyruvic acid content on blood.
    Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine (New York, N.Y.), 1952, Volume: 81, Issue:3

    Topics: Blood; Humans; Intellectual Disability; Phenylketonurias; Phenylpyruvic Acids

1952
Phenylketonuria VIII. Relation between age, serum phenylalanine level, and phenylpyruvic acid excretion.
    Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine (New York, N.Y.), 1957, Volume: 94, Issue:1

    Topics: Biochemical Phenomena; Biological Transport; Body Fluids; Humans; Phenylalanine; Phenylketonurias; P

1957
Phenylketonuria VIII. Relation between age, serum phenylalanine level, and phenylpyruvic acid excretion.
    Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine (New York, N.Y.), 1957, Volume: 94, Issue:1

    Topics: Biochemical Phenomena; Biological Transport; Body Fluids; Humans; Phenylalanine; Phenylketonurias; P

1957
Phenylketonuria VIII. Relation between age, serum phenylalanine level, and phenylpyruvic acid excretion.
    Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine (New York, N.Y.), 1957, Volume: 94, Issue:1

    Topics: Biochemical Phenomena; Biological Transport; Body Fluids; Humans; Phenylalanine; Phenylketonurias; P

1957
Phenylketonuria VIII. Relation between age, serum phenylalanine level, and phenylpyruvic acid excretion.
    Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine (New York, N.Y.), 1957, Volume: 94, Issue:1

    Topics: Biochemical Phenomena; Biological Transport; Body Fluids; Humans; Phenylalanine; Phenylketonurias; P

1957
Pathogenetic problems in phenylketonuria.
    The American journal of medicine, 1957, Volume: 22, Issue:5

    Topics: Phenylketonurias; Phenylpyruvic Acids

1957
Determination of phenylpyruvic acid in the urine of patients with oligophrenia phenylpyruvica.
    Clinica chimica acta; international journal of clinical chemistry, 1957, Volume: 2, Issue:5

    Topics: Body Fluids; Humans; Phenylketonurias; Phenylpyruvic Acids

1957
Excretion of phenylalanine; report of two cases of phenyl pyruvic acid oligophrenia.
    Annales paediatriae Fenniae, 1957, Volume: 3, Issue:4

    Topics: Biological Transport; Humans; Phenylalanine; Phenylketonurias; Phenylpyruvic Acids; Pyruvic Acid

1957
[Influence of fructose on phenylpyruvic acid excretion in phenylpyruvic oligophrenia].
    Klinische Wochenschrift, 1957, Jul-15, Volume: 35, Issue:14

    Topics: Biological Transport; Body Fluids; Fructose; Leadership; Phenylketonurias; Phenylpyruvic Acids

1957
[Infra-red spectroscopy of phenylpyruvic acid in urine of phenylketonuric patients].
    Revue de pathologie generale et de physiologie clinique, 1958, Volume: 58, Issue:694

    Topics: Body Fluids; Humans; Phenylketonurias; Phenylpyruvic Acids; Spectrum Analysis

1958
An inborn error of metabolism with the urinary excretion of alpha-hydroxy-butyric acid and phenylpyruvic acid.
    Archives of disease in childhood, 1958, Volume: 33, Issue:168

    Topics: Butyrates; Humans; Ketone Bodies; Phenylketonurias; Phenylpyruvic Acids

1958
[Phenylpyruvic acid oligophrenia].
    Neue osterreichische Zeitschrift fur Kinderheilkunde, 1958, Volume: 3, Issue:2-3

    Topics: Medical Records; Phenylketonurias; Phenylpyruvic Acids

1958
A method for the estimation of phenylpyruvic acid in urine with some examples of its use in dietary treatment of phenylpyruvic oligophrenia.
    Scandinavian journal of clinical and laboratory investigation, 1958, Volume: 10, Issue:4

    Topics: Diet; Humans; Phenylketonurias; Phenylpyruvic Acids; Regression Analysis

1958
Phenylpyruvic acid as a possible precursor of o-hydroxyphenylacetic acid in man.
    Science (New York, N.Y.), 1959, Jun-05, Volume: 129, Issue:3362

    Topics: Administration, Oral; Humans; Male; Phenylacetates; Phenylalanine; Phenylketonurias; Phenylpyruvic A

1959
Tests for phenylketonuria.
    Cerebral palsy bulletin, 1961, Volume: 3

    Topics: Body Fluids; Humans; Phenylketonurias; Phenylpyruvic Acids

1961
[Determination of phenylalanine and phenylpyruvic acid in the blood and urine. Application to two cases of phenylpyruvic oligophrenia].
    Helvetica paediatrica acta, 1959, Volume: 14

    Topics: Body Fluids; Humans; Phenylalanine; Phenylketonurias; Phenylpyruvic Acids

1959
[Phenylpyruvic oligophrenia. Detection and semiquantitative determination of phenylpyruvic acid in the urine with the help of phenistix].
    Nordisk medicin, 1960, Jul-07, Volume: 64

    Topics: Body Fluids; Humans; Phenylketonurias; Phenylpyruvic Acids

1960
Problems of routine screening for phenylketonuria.
    Lancet (London, England), 1962, Sep-08, Volume: 2, Issue:7254

    Topics: Humans; Phenylalanine; Phenylketonurias; Phenylpyruvic Acids

1962
The renal clearance of phenylpyruvate.
    Clinica chimica acta; international journal of clinical chemistry, 1961, Volume: 6

    Topics: Humans; Kidney; Phenylketonurias; Phenylpyruvic Acids

1961
[Clinico-genetic studies in phenylpyruvic acid feeblemindedness].
    Arztliche Forschung, 1963, Jan-10, Volume: 17

    Topics: Chromosomes; Phenylketonurias; Phenylpyruvic Acids; Research

1963
[Identification and determination of phenylpyruvic acid in the urine].
    Medecine et laboratoire, 1963, Volume: 16

    Topics: Body Fluids; Humans; Phenylketonurias; Phenylpyruvic Acids

1963
[Contribution to determination of alpha-keto acids in the urine, with special reference to phenylpyruvic acid].
    Wiener klinische Wochenschrift, 1962, Sep-21, Volume: 74

    Topics: Humans; Keto Acids; Phenylketonurias; Phenylpyruvic Acids

1962
[PHENYLKETONURIA. APROPOS OF A FAMILIAL CASE].
    Pediatrie, 1963, Volume: 18

    Topics: Blood Chemical Analysis; Body Fluids; Diet; Diet Therapy; Electroencephalography; Genetics, Medical;

1963
A STUDY OF THE INCIDENCE OF PHENYLKETONURIA IN INDIA.
    The Indian journal of medical research, 1963, Volume: 51

    Topics: Blood; Consanguinity; Epidemiology; Family; Humans; Incidence; India; Infant; Infant, Newborn; Pheny

1963
THE INDUCTION OF TYROSYLURIA IN YOUNG RATS.
    Annals of the New York Academy of Sciences, 1963, Dec-30, Volume: 111

    Topics: Animals; Ascorbic Acid; Guinea Pigs; Metabolism; Phenylketonurias; Phenylpyruvic Acids; Rats; Resear

1963
[RECENT KNOWLEDGE ABOUT THE PROBLEM OF PHENYLPYRUVIC OLIGOPHRENIA. FREQUENCY, GENETICS, BIOCHEMISTRY, DIAGNOSIS, AND THERAPEUTICS].
    La Semana medica, 1963, Oct-14, Volume: 123

    Topics: Asparagine; Biochemistry; Diet; Diet Therapy; Genetics, Medical; Glutamine; Humans; Knowledge; Niala

1963
PHENYLKETONURIA IN A SUDANESE FAMILY.
    The Journal of pediatrics, 1964, Volume: 64

    Topics: Africa; Africa, Central; Black People; Blood Chemical Analysis; Child; Chromatography; Genetics, Med

1964
URINARY PHENYLPYRUVIC ACID IN PHENYLKETONURIA.
    JAMA, 1964, May-25, Volume: 188

    Topics: Blood Chemical Analysis; Chlorides; Chromatography; Humans; Infant; Infant, Newborn; Iron; Phenylala

1964
SEROTONIN DEFICIENCY IN INFANCY AS ONE CAUSE OF A MENTAL DEFECT IN PHENYLKETONURIA.
    Science (New York, N.Y.), 1964, May-15, Volume: 144, Issue:3620

    Topics: Animals; Animals, Newborn; Behavior; Catecholamines; Chlorpromazine; Genetics; Learning; Metabolic D

1964
AMINO ACID METABOLISM IN KWASHIORKOR. II. METABOLISM OF PHENYLALANINE AND TYROSINE.
    Clinical science, 1964, Volume: 26

    Topics: Blood Chemical Analysis; Child; Chromatography; Dietary Proteins; Humans; Infant; Kwashiorkor; Milk;

1964
[METABOLIC ASPECTS OF PHENYLKETONURIA WITH PARTICULAR ATTENTION TO URINARY ELIMINATION OF SOME PHENOLIC ACIDS].
    Minerva pediatrica, 1964, Sep-15, Volume: 16

    Topics: Adolescent; Attention; Biomedical Research; Child; Humans; Hydroxybenzoates; Indoleacetic Acids; Man

1964
CHEMICAL DIAGNOSIS OF PHENYLKETONURIA. REPORT OF 8 CASES.
    Chinese medical journal (Peking, China : 1932), 1964, Volume: 83

    Topics: Adolescent; Blood Chemical Analysis; Body Fluids; Child; China; Chromatography; Clinical Laboratory

1964
LEVOMEPROMAZINE (NOZINAN) MEDICATION AS A SOURCE OF ERROR IN THE LABORATORY DIAGNOSIS OF PHENYLKETONURIA.
    Scandinavian journal of clinical and laboratory investigation, 1965, Volume: 17

    Topics: Blood; Chlorides; Clinical Laboratory Techniques; Diagnosis; Humans; Indicators and Reagents; Methot

1965
An improved test for phenylketonuria.
    Clinical chemistry, 1959, Volume: 5

    Topics: Humans; Phenylketonurias; Phenylpyruvic Acids

1959
The galactose tolerance test in phenylketonuria.
    The Journal of mental science, 1950, Volume: 96, Issue:404

    Topics: Body Fluids; Galactose; Humans; Immune Tolerance; Liver; Phenylketonurias; Phenylpyruvic Acids; Urin

1950
Measurement of pleiotropic effects in phenylketonuria.
    Annals of eugenics, 1951, Volume: 16, Issue:2

    Topics: Phenylketonurias; Phenylpyruvic Acids

1951
Renal transport of aromatic acids in patients with phenylketonuria.
    Journal of inherited metabolic disease, 1981, Volume: 4, Issue:2

    Topics: Child; Humans; Kidney; Metabolic Clearance Rate; Phenylketonurias; Phenylpyruvic Acids

1981
Phenylpyruvate, fetal damage, and maternal phenylketonuria syndrome.
    Lancet (London, England), 1993, May-22, Volume: 341, Issue:8856

    Topics: Embryonic and Fetal Development; Female; Humans; Infant, Newborn; Phenylketonurias; Phenylpyruvic Ac

1993
Phenylalanine determined in plasma with use of phenylalanine dehydrogenase and a centrifugal analyzer.
    Clinical chemistry, 1993, Volume: 39, Issue:1

    Topics: Amino Acid Oxidoreductases; Autoanalysis; Humans; NAD; Phenylalanine; Phenylketonurias; Phenylpyruvi

1993
Measurement of phenyllactate, phenylacetate, and phenylpyruvate by negative ion chemical ionization-gas chromatography/mass spectrometry in brain of mouse genetic models of phenylketonuria and non-phenylketonuria hyperphenylalaninemia.
    Analytical biochemistry, 2000, May-01, Volume: 280, Issue:2

    Topics: Animals; Brain; Disease Models, Animal; Gas Chromatography-Mass Spectrometry; Humans; Lactates; Mice

2000
Metabolism of carnitine in phenylacetic acid-treated rats and in patients with phenylketonuria.
    Biochimica et biophysica acta, 2000, Jun-15, Volume: 1501, Issue:2-3

    Topics: Adult; Animals; Betaine; Carnitine; Female; Glutamic Acid; Homogentisic Acid; Humans; Ketoglutaric A

2000
Phenylalanine and phenylpyruvate inhibit ATP diphosphohydrolase from rat brain cortex.
    International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience, 2001, Volume: 19, Issue:7

    Topics: 5'-Nucleotidase; Adenosine; Adenosine Diphosphate; Adenosine Triphosphate; Animals; Apyrase; Cerebra

2001
A synopsis of the unconjugated acidic transamination metabolites of phenylalanine in phenylketonuria.
    Journal of inherited metabolic disease, 1992, Volume: 15, Issue:1

    Topics: Adolescent; Child; Child, Preschool; Female; Humans; Kidney; Lactates; Male; Phenylacetates; Phenyla

1992
Cobalt(II)-catalyzed chemiluminescence in a dioctadecyldimethylammonium chloride bilayer membranous medium for the flow injection determination of phenylpyruvic acid.
    Analytical chemistry, 1991, Oct-15, Volume: 63, Issue:20

    Topics: Adult; Cobalt; Flow Injection Analysis; Humans; Lipid Bilayers; Luminescent Measurements; Phenylketo

1991
Examination of urine metabolites in the newborn period and during protein loading tests at 6 months of age--Part 1.
    European journal of pediatrics, 1990, Volume: 149 Suppl 1

    Topics: Creatinine; Dietary Proteins; Humans; Infant, Newborn; Phenylacetates; Phenylketonurias; Phenylpyruv

1990
Excretion of phenylpyruvic, 4-hydroxyphenylpyruvic and indolyl-3-acetic acids by the skin fibroblasts from a phenylketonuric child.
    Journal of inherited metabolic disease, 1988, Volume: 11, Issue:3

    Topics: Adult; Child, Preschool; Epidermis; Female; Fibroblasts; Humans; Indoleacetic Acids; Phenylketonuria

1988
Gas chromatographic analysis of abnormal urinary organic acids in phenylketonuria.
    Taiwan yi xue hui za zhi. Journal of the Formosan Medical Association, 1985, Volume: 84, Issue:11

    Topics: Adolescent; Adult; Child; Child, Preschool; Chromatography, Gas; Female; Humans; Lactates; Male; Phe

1985
Phenylalanine metabolites, attention span and hyperactivity.
    The American journal of clinical nutrition, 1985, Volume: 42, Issue:2

    Topics: Adolescent; Attention; Child; Child, Preschool; Female; Humans; Hyperkinesis; Infant; Infant, Newbor

1985