fenclonine and Phenylketonurias studies

Fenclonine: A selective and irreversible inhibitor of tryptophan hydroxylase, a rate-limiting enzyme in the biosynthesis of serotonin (5-HYDROXYTRYPTAMINE). Fenclonine acts pharmacologically to deplete endogenous levels of serotonin.

Phenylketonurias: A group of autosomal recessive disorders marked by a deficiency of the hepatic enzyme PHENYLALANINE HYDROXYLASE or less frequently by reduced activity of DIHYDROPTERIDINE REDUCTASE (i.e., atypical phenylketonuria). Classical phenylketonuria is caused by a severe deficiency of phenylalanine hydroxylase and presents in infancy with developmental delay; SEIZURES; skin HYPOPIGMENTATION; ECZEMA; and demyelination in the central nervous system. (From Adams et al., Principles of Neurology, 6th ed, p952).

Research Excerpts

Excerpt	Relevance	Reference
"Infant rhesus monkeys were (a) fed a diet high in phenylalanine or (b) fed a diet high in p-chlorophenylalanine for the 1st 12 months of life, (c) selected from mothers fed a diet high in phenylalanine during pregnancy, (d) fed a diet low in phenylalanine, (e) maintained as controls, or (f) maintained as pair-fed controls."	3.66	Dietary and metabolic effects on rhesus social behavior: phenylalanine-related dietary alterations. ( Chamove, AS, 1980)
" Increased concentrations of phenylalanine and increased ratio of phenylalanine to tyrosine were demonstrated in blood from pregnant rats fed the experimental PKU diet from day 10 to 20 of pregnancy, in fetal blood and amniotic fluid of fetal animals from mothers fed the PKU diet, and in blood of rats fed the PKU diet for 28-30 days beginning at 20-21 days of age."	3.65	Biochemical effects of induced phenylketonuria in rats. ( Berry, HK; Butcher, RE; Kazmaier, KJ; Poncet, IB, 1975)
"Recent approaches for treating phenylketonuria focus on injectable medications that efficiently break down phenylalanine but sometimes result in detrimentally low phenylalanine levels."	1.51	Phenylalanine Monitoring via Aptamer-Field-Effect Transistor Sensors. ( Andrews, AM; Cheung, KM; Jung, ME; Nakatsuka, N; Stojanović, MN; Weiss, PS; Yang, H; Yang, KA; Ye, M; Zhao, C, 2019)
"Phenylketonuria is a genetic defect that leads to imbecility, if the diagnosis is not made directly after birth."	1.26	Phenylketonuria: clinical and experimental considerations revealed by the use of animal models. ( Lane, JD; Neuhoff, V, 1980)
"This degree of hyperphenylalaninaemia, persisting for 18h per day throughout a critical period of development, fulfils the primary criterion of a suitable animal model for phenylketonuria."	1.26	The regulation of phenylalanine hydroxylase in rat tissues in vivo. The maintenance of high plasma phenylalanine concentrations in suckling rats: a model for phenylketonuria. ( Delvalle, JA; Greengard, O, 1976)
"These manifestations of phenylketonuria were reproduced in rats treated with phenylacetate in amounts approximating those likely to be produced in phenylketonuria."	1.26	Myelin deficiency in experimental phenylketonuria: contribution of the aromatic acid metabolites of phenylalanine. ( Loo, YH; Scotto, J; Wisniewski, HM, 1978)
"Model phenylketonuria was induced in albino rats by injecting (at 1-20 days of age) and feeding (at 21-80 days) L-phenylalanine and D-L-para-chloro-phenylalanine."	1.25	Model phenylketonuria (PKU) in the albino rat: behaviroal, biochemical, and neuroanatomical effects. ( Brown, WJ; Copenhaver, JH; Gunter, R; Schalock, RL, 1975)
"An experimental phenylketonuria-like syndrome was produced in rats by oral administration of 1-phenylalanine (Phe, 500 mg/kg) and dl-p-chloro-phenylalanine (pCPA, 100-125 mg/kg) daily from the 2nd-3rd day of life to the age of 42 days."	1.25	Protective effect of tryptophan and 5-hydroxytryptophan on experimental phenylketonuria induced with phenylalanine+ p-cholorophenylalanine in rats. ( Airaksinen, MM; MacDonald, EJ; Marvola, M; Turakka, H, 1975)

Timeframe	Studies, this research(%)	All Research%
pre-1990	52 (94.55)	18.7374
1990's	0 (0.00)	18.2507
2000's	1 (1.82)	29.6817
2010's	2 (3.64)	24.3611
2020's	0 (0.00)	2.80

Authors	Studies
Cheung, KM	1
Yang, KA	1
Nakatsuka, N	1
Zhao, C	1
Ye, M	1
Jung, ME	1
Yang, H	1
Weiss, PS	1
Stojanović, MN	1
Andrews, AM	1
Simon, KR	1
Dos Santos, RM	1
Scaini, G	1
Leffa, DD	1
Damiani, AP	1
Furlanetto, CB	1
Machado, JL	1
Cararo, JH	1
Macan, TP	1
Streck, EL	2
Ferreira, GC	1
Andrade, VM	1
Schuck, PF	1
Loo, YH	5
Fulton, T	1
Miller, K	1
Wisniewski, HM	2
Chamove, AS	2
Benevenga, NJ	1
Steele, RD	1
Dienel, GA	1
Dwivedy, AK	1
Shah, SN	1
Lane, JD	2
Schöne, B	1
Langenbeck, U	1
Neuhoff, V	2
Fulton, TR	1
Triano, T	1
Rabe, A	1
Lo, GS	1
Longenecker, JB	1
Vorhees, CV	3
Butcher, RE	3
Berry, HK	4
Zanić-Grubisić, T	1
Lipovac, K	1
Sharma, SD	1
Cooley, C	1
De La Pava, E	1
Ambrus, CM	1
Edom, PT	1
Noriler, ME	1
Borges, LF	1
Pontes, ZL	1
Parolo, E	1
Dutra-Filho, CS	1
Wannmacher, CM	1
Wyse, AT	1
Murthy, LT	1
Avins, L	1
Guroff, G	3
Kuwabara, T	1
Brase, DA	1
Loh, HH	1
Valdivieso, F	4
Gimenez, C	4
Mayor, F	4
Rowe, VD	1
Fales, HM	1
Pisano, JJ	1
Andersen, AE	2
Poncet, IB	1
Kazmaier, KJ	1
Antonas, KN	1
Coulson, WF	1
Schalock, RL	4
Brown, WJ	3
Copenhaver, JH	4
Gunter, R	2
Airaksinen, MM	1
Turakka, H	1
Marvola, M	1
MacDonald, EJ	1
Delvalle, JA	1
Greengard, O	1
Luchins, D	1
Dhondt, JL	2
Farriaux, JP	2
Ugarte, M	1
Maties, M	1
Dautrevaux, M	1
Biserte, G	1
Scotto, J	1
Akeson, AL	1
Brunner, RL	1
Lasala, JM	1
Coscia, CJ	1
Benavides, J	1
Sanchez-Rubiales, M	1
Okano, Y	1
Chow, IZ	1
Isshiki, G	1
Inoue, A	1
Oura, T	1
Ledley, FD	1
Woo, SL	1
Pueschel, SM	1
Boylan, J	1
Ellenbogen, R	1
Kilbey, MM	1
Harris, RT	1
Presant, CA	1
Wiernik, PH	1
Serpick, AA	1
Schalock, R	1
Fuller, RW	1
Snoddy, HD	1
Wolen, RL	1
Coburn, SP	1
Sirlin, EM	1
Mack, K	2
Shihabi, ZK	1
Summer, GK	1
Edwards, DJ	2
Blau, K	2
Vacanti, JP	1
Carver, MJ	2
Gunter, RG	1
Tourian, A	1

Article	Year
Adverse effects of excessive consumption of amino acids. Annual review of nutrition, 1984, Volume: 4 Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Animals; Brain; Cattle; Diet; Disease Models, Ani	1984
Progress in experimental phenylketonuria: a critical review. Neuroscience and biobehavioral reviews, 1981,Spring, Volume: 5, Issue:1 Topics: Abnormalities, Multiple; Animals; Disease Models, Animal; Energy Metabolism; Female; Fenclonine; Glu	1981
Urinary phenylalanine metabolites in preweanling rats treated with p-chlorophenylalanine and phenylalanine. Nutrition reviews, 1975, Volume: 33, Issue:9 Topics: Animals; Disease Models, Animal; Fenclonine; Humans; Lactates; Phenylalanine; Phenylketonurias; Phen	1975
Biogenic amines and affective disorders. A critical analysis. International pharmacopsychiatry, 1976, Volume: 11, Issue:3 Topics: 5-Hydroxytryptophan; Affective Symptoms; Antidepressive Agents, Tricyclic; Biogenic Amines; Bipolar	1976

Article	Year
Phenylalanine Monitoring via Aptamer-Field-Effect Transistor Sensors. ACS sensors, 2019, 12-27, Volume: 4, Issue:12 Topics: Animals; Aptamers, Nucleotide; DNA; Electrochemical Techniques; Fenclonine; Mice; Phenylalanine; Phe	2019
DNA damage induced by phenylalanine and its analogue p-chlorophenylalanine in blood and brain of rats subjected to a model of hyperphenylalaninemia. Biochemistry and cell biology = Biochimie et biologie cellulaire, 2013, Volume: 91, Issue:5 Topics: Animals; Brain; DNA Damage; Fenclonine; Male; Phenylalanine; Phenylalanine Hydroxylase; Phenylketonu	2013
Phenylacetate and brain dysfunction in experimental phenylketonuria: synaptic development. Life sciences, 1980, Oct-06, Volume: 27, Issue:14 Topics: Animals; Cerebral Cortex; Choline; Female; Fenclonine; gamma-Aminobutyric Acid; Humans; Male; Neurot	1980
Analysis of learning in retarded monkeys. Journal of mental deficiency research, 1984, Volume: 28 (Pt 1) Topics: Animals; Attention; Concept Formation; Discrimination, Psychological; Disease Models, Animal; Female	1984
Chronic hyperphenylalaninemia produces cerebral hyperglycinemia in immature rats. Journal of neurochemistry, 1981, Volume: 36, Issue:1 Topics: Amino Acids; Animals; Animals, Newborn; Brain; Fenclonine; Glycine; Humans; Liver; Phenylalanine; Ph	1981
Effect of hyperphenylalaninemia on polyphosphoinositides content of rat brain. Experientia, 1982, Dec-15, Volume: 38, Issue:12 Topics: Animals; Brain; Disease Models, Animal; Fenclonine; Humans; Kinetics; Phenylalanine; Phenylketonuria	1982
Characterization of experimental phenylketonuria. Augmentation of hyperphenylalaninemia with alpha-methylphenylalanine and p-chlorophenylalanine. Biochimica et biophysica acta, 1980, Jan-17, Volume: 627, Issue:2 Topics: Age Factors; Amino Acids; Animals; Behavior, Animal; Body Weight; Brain Chemistry; Disease Models, A	1980
Dietary and metabolic effects on rhesus social behavior: phenylalanine-related dietary alterations. Developmental psychobiology, 1980, Volume: 13, Issue:3 Topics: Aggression; Animals; Female; Fenclonine; Haplorhini; Humans; Macaca mulatta; Phenylalanine; Phenylke	1980
Phenylketonuria: clinical and experimental considerations revealed by the use of animal models. Die Naturwissenschaften, 1980, Volume: 67, Issue:5 Topics: Animals; Brain; Cerebellum; Disease Models, Animal; Fenclonine; Humans; Male; Neurons; Phenylalanine	1980
Phenylacetate and the enduring behavioral deficit in experimental phenylketonuria. Life sciences, 1980, Oct-06, Volume: 27, Issue:14 Topics: Animals; Behavior, Animal; Body Weight; Escape Reaction; Female; Fenclonine; Humans; Hyperkinesis; M	1980
Induction of an experimental phenylketonuria-like condition in infant rats during the first 2 weeks after birth. The American journal of clinical nutrition, 1981, Volume: 34, Issue:4 Topics: Animals; Animals, Newborn; Diet; Disease Models, Animal; Female; Fenclonine; Humans; Male; Methotrex	1981
Disturbances of amino acid transport in rats with experimental hyperphenylalaninaemia. Journal of inherited metabolic disease, 1981, Volume: 4, Issue:2 Topics: Amino Acids; Animals; Cerebral Cortex; Disease Models, Animal; Fenclonine; Glycine; Humans; Leucine;	1981
Development of a refractory stage in a dog model for phenylketonuria. Research communications in chemical pathology and pharmacology, 1981, Volume: 33, Issue:1 Topics: Animals; Diet; Disease Models, Animal; Dogs; Fenclonine; Humans; Liver; Phenylalanine Hydroxylase; P	1981
Effect of phenylalanine and p-chlorophenylalanine on Na+, K+-ATPase activity in the synaptic plasma membrane from the cerebral cortex of rats. Metabolic brain disease, 2000, Volume: 15, Issue:2 Topics: Animals; Animals, Newborn; Body Weight; Ca(2+) Mg(2+)-ATPase; Cerebral Cortex; Fenclonine; Nerve Tis	2000
Inhibition of phenylalanine hydroxylase activity by alpha-methyl tyrosine, a potent inhibitor of tyrosine hydroxylase. Life sciences, 1975, Dec-15, Volume: 17, Issue:12 Topics: Animals; Disease Models, Animal; Fenclonine; Humans; Kidney; Liver; Methyltyrosines; Mice; Phenylala	1975
Ultrastructural changes in rat optic nerve associated with hyperphenylalaninemia induced by para-chlorophenylalanine and phenylalanine. Journal of neuropathology and experimental neurology, 1975, Volume: 34, Issue:2 Topics: Animals; Astrocytes; Axons; Cytoplasm; Disease Models, Animal; Endoplasmic Reticulum; Fenclonine; Go	1975
Possible role of 5-hydroxyptamine in minimal brain dysfunction. Life sciences, 1975, Apr-01, Volume: 16, Issue:7 Topics: Amphetamine; Animal Nutritional Physiological Phenomena; Animals; Attention Deficit Disorder with Hy	1975
In vivo inhibition of rat liver phenylalanine hydroxylase by p-chlorophenylalanine and Esculin. Experimental model of phenylketonuria. Biochemical medicine, 1975, Volume: 12, Issue:1 Topics: Animals; Disease Models, Animal; Esculin; Female; Fenclonine; Flavonoids; Humans; Liver; Phenylalani	1975
Urinary metabolites of phenylalanine in the preweanling rat treated with p-chlorophenylalanine and phenylalanine. Biochemical medicine, 1975, Volume: 12, Issue:2 Topics: Animals; Chromatography; Disease Models, Animal; Dose-Response Relationship, Drug; Female; Fenclonin	1975
Biochemical effects of induced phenylketonuria in rats. Biology of the neonate, 1975, Volume: 26, Issue:1-2 Topics: Amino Acids; Amniotic Fluid; Animals; Brain; Diet; Female; Fenclonine; Fetal Blood; Humans; Phenylac	1975
Brain uptake and protein incorporation of amino acids studied in rats subjected to prolonged hyperphenylalaninaemia. Journal of neurochemistry, 1975, Volume: 25, Issue:3 Topics: Amino Acids; Animals; Brain; Disease Models, Animal; Female; Fenclonine; Glycine; Humans; Leucine; L	1975
Model phenylketonuria (PKU) in the albino rat: behaviroal, biochemical, and neuroanatomical effects. Journal of comparative and physiological psychology, 1975, Volume: 89, Issue:6 Topics: Animals; Axons; Body Weight; Disease Models, Animal; Emotions; Fenclonine; Humans; Learning; Liver;	1975
Protective effect of tryptophan and 5-hydroxytryptophan on experimental phenylketonuria induced with phenylalanine+ p-cholorophenylalanine in rats. Medical biology, 1975, Volume: 53, Issue:6 Topics: 5-Hydroxytryptophan; Animals; Behavior, Animal; Body Weight; Brain; Female; Fenclonine; Humans; Hydr	1975
The regulation of phenylalanine hydroxylase in rat tissues in vivo. The maintenance of high plasma phenylalanine concentrations in suckling rats: a model for phenylketonuria. The Biochemical journal, 1976, Mar-15, Volume: 154, Issue:3 Topics: Animals; Brain; Disease Models, Animal; Fenclonine; Humans; Kidney; Liver; Male; Phenylalanine; Phen	1976
Study of blood aminoacids in experimental hyperphenylalaninemia. Biomedicine / [publiee pour l'A.A.I.C.I.G.], 1977, Volume: 27, Issue:2 Topics: Amino Acids; Animals; Disease Models, Animal; Fenclonine; Humans; Male; Phenylalanine; Phenylketonur	1977
Free amino acids in the tissues of rats with experimentally induced phenylketonuria. Journal of mental deficiency research, 1977, Volume: 21, Issue:2 Topics: Amino Acids; Animals; Brain; Brain Chemistry; Esculin; Fenclonine; Humans; Liver; Male; Phenylalanin	1977
A new experimental model of hyperphenylalaninemia in rat. Effect of p-chlorophenylalanine and cotrimoxazole. Biochimie, 1977, Volume: 59, Issue:8-9 Topics: Animals; Disease Models, Animal; Drug Combinations; Fenclonine; Humans; Kidney; Liver; Male; Phenyla	1977
Myelin deficiency in experimental phenylketonuria: contribution of the aromatic acid metabolites of phenylalanine. Advances in experimental medicine and biology, 1978, Volume: 100 Topics: Animals; Brain; Disease Models, Animal; Female; Fenclonine; Humans; Male; Membrane Lipids; Myelin Pr	1978
Brain pyruvate kinase activity in PKU model systems. Journal of neurochemistry, 1979, Volume: 32, Issue:1 Topics: Animals; Body Weight; Brain; Female; Fenclonine; Fetus; Humans; Organ Size; Phenylalanine; Phenylket	1979
Accumulation of a tetrahydroisoquinoline in phenylketonuria. Science (New York, N.Y.), 1979, Jan-19, Volume: 203, Issue:4377 Topics: Adolescent; Animals; Brain; Child; Child, Preschool; Dopamine beta-Hydroxylase; Fenclonine; Humans;	1979
Experimental phenylketonuria: metabolic studies in rat liver. Molecular and cellular biochemistry, 1977, May-31, Volume: 16, Issue:1 Topics: Animals; Disease Models, Animal; Fasting; Female; Fenclonine; Fructosephosphates; Gluconeogenesis; G	1977
Effects of phenylalanine loading on protein synthesis in the fetal heart and brain of rat: an experimental approach to maternal phenylketonuria. Journal of inherited metabolic disease, 1986, Volume: 9, Issue:1 Topics: Animals; Brain; Carbon Radioisotopes; Disease Models, Animal; Female; Fenclonine; Fetal Heart; Fetus	1986
P-chlorophenylalanine does not inhibit production of recombinant human phenylalanine hydroxylase in NIH3T3 cells or E. coli. Biochemical and biophysical research communications, 1987, Jan-30, Volume: 142, Issue:2 Topics: Animals; Cell Line; Escherichia coli; Fenclonine; Humans; Mice; Phenylalanine Hydroxylase; Phenylket	1987
Studies on experimentally induced hyperphenylalaninemia. Journal of mental deficiency research, 1988, Volume: 32 ( Pt 4) Topics: Animals; Disease Models, Animal; Drug Combinations; Female; Fenclonine; Phenylalanine; Phenylalanine	1988
Behavioral, biochemical and maturation effects of early DL-para-chlorophenylalanine treatment. Psychopharmacologia, 1971, Volume: 19, Issue:4 Topics: Animals; Behavior, Animal; Body Weight; Bone Development; Brain Chemistry; Female; Fenclonine; Human	1971
Effects of phenylalanine and related compounds on serum alkaline phosphatase assay. Clinica chimica acta; international journal of clinical chemistry, 1971, Volume: 32, Issue:3 Topics: Adult; Alkaline Phosphatase; Bone Diseases; Chemistry, Clinical; Chloramphenicol; Fenclonine; Humans	1971
Enduring behavioral changes in rats with experimental phenylketonuria. Proceedings of the National Academy of Sciences of the United States of America, 1972, Volume: 69, Issue:4 Topics: Aggression; Animals; Appetitive Behavior; Avoidance Learning; Behavior, Animal; Brain; Defecation; D	1972
Cataractogenesis in infant rats with model phenylketonuria. Perceptual and motor skills, 1972, Volume: 35, Issue:1 Topics: Age Factors; Animals; Animals, Newborn; Cataract; Disease Models, Animal; Female; Fenclonine; Humans	1972
Effect of glucagon and p-chlorophenylalanine on hepatic enzymes that metabolize phenylalanine. Advances in enzyme regulation, 1972, Volume: 10 Topics: Adolescent; Adult; Animals; Fenclonine; Glucagon; Humans; Liver; Male; Middle Aged; Phenylalanine; P	1972
Effect of hyperphenylalaninemia on vitamin B 6 metabolism in developing rat brain. Journal of neurochemistry, 1972, Volume: 19, Issue:10 Topics: Animals; Brain; Carbon Isotopes; Chromatography, Ion Exchange; Female; Fenclonine; Humans; Injection	1972
Effect of vitamin B 6 on phenylalanine metabolism in the brain of normal and p-chlorophenylalanine-treated rats. Journal of neurochemistry, 1972, Volume: 19, Issue:10 Topics: Animals; Brain; Carbon Isotopes; Female; Fenclonine; Humans; Injections, Intraperitoneal; Injections	1972
Determination of serum and urinary phenylalanine by gas chromatography. Clinical chemistry, 1973, Volume: 19, Issue:5 Topics: Amino Acids; Chromatography, Gas; Fenclonine; Humans; Lactates; Phenylalanine; Phenylketonurias	1973
Aromatic acids derived from phenylalanine in the tissues of rats with experimentally induced phenylketonuria-like characteristics. The Biochemical journal, 1972, Volume: 130, Issue:2 Topics: Animals; Benzoates; Brain; Chromatography, Gas; Fenclonine; Humans; Lactates; Liver; Male; Mandelic	1972
Phenethylamines in brain and liver of rats with experimentally induced phenylketonuria-like characteristics. The Biochemical journal, 1973, Volume: 132, Issue:1 Topics: Animals; Brain; Centrifugation, Density Gradient; Chromatography, Gas; Ethanolamines; Fenclonine; Hu	1973
Experimental maternal hyperphenylalaninemia: disaggregation of fetal brain ribosomes. Journal of neurochemistry, 1973, Volume: 21, Issue:2 Topics: Animals; Brain; Brain Chemistry; Centrifugation, Density Gradient; Female; Fenclonine; Fetus; Humans	1973
The effects of parachlorophenylalanine on lens ultrastructure. Experimental eye research, 1973, Nov-11, Volume: 17, Issue:3 Topics: Animals; Cataract; Cell Nucleus; Cytoplasm; Endoplasmic Reticulum; Epithelial Cells; Female; Fenclon	1973
Behavioral effects of experimental maternal hyperphenylalaninemia. Developmental psychobiology, 1973, Volume: 6, Issue:6 Topics: Animals; Animals, Newborn; Avoidance Learning; Behavior, Animal; Disease Models, Animal; Exploratory	1973
Experimental maternal hyperphenylalaninemia: biochemical effects and offspring development. Developmental psychobiology, 1974, Volume: 7, Issue:2 Topics: Aggression; Animals; Animals, Newborn; Body Weight; Brain; Brain Chemistry; Disease Models, Animal;	1974
Reduced activity in rats with induced phenylketonuria. Developmental psychobiology, 1972, Volume: 5, Issue:2 Topics: Animals; Body Weight; Diet; Eating; Exploratory Behavior; Fenclonine; Humans; Male; Motor Activity;	1972
Glycolysis in the brain and liver of rats with experimentally induced phenylketonuria. Biochemical medicine, 1974, Volume: 11, Issue:1 Topics: Animals; Autoanalysis; Brain; Chromatography, Ion Exchange; Esculin; Female; Fenclonine; Glycolysis;	1974
Control of liver and brain aromatic amino-acid metabolism by phenylalanine hydroxylase. Voprosy biokhimii mozga, 1973, Volume: 8 Topics: Animals; Brain; Brain Chemistry; Chemical Phenomena; Chemistry; Disease Models, Animal; Electrophore	1973

fenclonine and Phenylketonurias

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