Page last updated: 2024-11-08

alanine and BH4 Deficiency

alanine has been researched along with BH4 Deficiency in 19 studies

Alanine: A non-essential amino acid that occurs in high levels in its free state in plasma. It is produced from pyruvate by transamination. It is involved in sugar and acid metabolism, increases IMMUNITY, and provides energy for muscle tissue, BRAIN, and the CENTRAL NERVOUS SYSTEM.
alanine : An alpha-amino acid that consists of propionic acid bearing an amino substituent at position 2.

Research Excerpts

ExcerptRelevanceReference
"21 individuals with phenylketonuria (ages 6 to 28 years, mean 11."2.69Randomised controlled trial of tyrosine supplementation on neuropsychological performance in phenylketonuria. ( Austin, V; Clarke, JT; Hanley, WB; Klim, P; Lehotay, DC; Schoonheyt, W; Smith, ML, 1998)

Research

Studies (19)

TimeframeStudies, this research(%)All Research%
pre-199013 (68.42)18.7374
1990's3 (15.79)18.2507
2000's2 (10.53)29.6817
2010's1 (5.26)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Tansek, MZ1
Groselj, U1
Kelvisar, M1
Kobe, H1
Lampret, BR1
Battelino, T1
Ambrose, JA1
Ingerson, A1
Garrettson, LG1
Chung, CW1
Brown, KJ2
Vesey, BV1
Tannock, GW1
Bell, EB1
Lines, DR2
Elliott, RB1
Waters, PI1
Hewson, AS1
Scriver, CR1
Treacy, EP1
Martinez, A1
Knappskog, PM1
Parniak, MA1
Smith, ML1
Hanley, WB1
Clarke, JT1
Klim, P1
Schoonheyt, W1
Austin, V1
Lehotay, DC1
Wyse, AT2
Noriler, ME1
Borges, LF1
Floriano, PJ1
Silva, CG1
Wajner, M2
Wannmacher, CM3
Carreras, AL1
de Mattos-Dutra, A1
Meirelles, R1
da Rocha, BB1
Pessoa-Pureur, R1
Bedin, M1
Estrella, CH1
Duarte, DV1
Ponzi, D1
Dutra-Filho, CS1
Greco, GM1
Magli, A1
Thalhammer, O1
Waisman, HA1
Chamove, AS1
Kerr, GR1
Harlow, HF1
Kamarýt, J1
Mrskos, A1
François, J1
Sietti, C1
Gruemer, HD1
Grannis, GF1
Hetland, LB1
Costantini, ML1
Clayton, BE1
Heeley, AF1
Heeley, M1

Reviews

3 reviews available for alanine and BH4 Deficiency

ArticleYear
[Eye manifestations of amino acid disorders].
    Minerva pediatrica, 1978, May-31, Volume: 30, Issue:10

    Topics: Alanine; Albinism; Alkaptonuria; Amino Acid Metabolism, Inborn Errors; Child; Cystinosis; Cystinuria

1978
Ocular manifestations in aminoacidopathies.
    Advances in ophthalmology = Fortschritte der Augenheilkunde = Progres en ophtalmologie, 1972, Volume: 25

    Topics: Adult; Alanine; Albinism; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Cystinosis;

1972
Amino acid intoxication.
    Food and cosmetics toxicology, 1971, Volume: 9, Issue:1

    Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Animals; Benzyl Compounds; Biological Tr

1971

Trials

1 trial available for alanine and BH4 Deficiency

ArticleYear
Randomised controlled trial of tyrosine supplementation on neuropsychological performance in phenylketonuria.
    Archives of disease in childhood, 1998, Volume: 78, Issue:2

    Topics: Administration, Oral; Adolescent; Adult; Alanine; Child; Cross-Over Studies; Double-Blind Method; Fe

1998

Other Studies

15 other studies available for alanine and BH4 Deficiency

ArticleYear
Long-term BH4 (sapropterin) treatment of children with hyperphenylalaninemia - effect on median Phe/Tyr ratios.
    Journal of pediatric endocrinology & metabolism : JPEM, 2016, May-01, Volume: 29, Issue:5

    Topics: Alanine; Biomarkers; Biopterins; Child; Child, Preschool; Diet; Female; Follow-Up Studies; Humans; I

2016
A study of the fluorometric method for phenylalanine in serum samples.
    Clinica chimica acta; international journal of clinical chemistry, 1967, Volume: 15, Issue:3

    Topics: Adult; Alanine; Amino Acids; Buffers; Child; Child, Preschool; Dipeptides; Female; Filtration; Fluor

1967
The assay on a defined medium of the effects of beta-2-thienylalanine on the growth of anaerobic bacterial isolates from phenylketonuric patients.
    Medical microbiology and immunology, 1980, Volume: 168, Issue:1

    Topics: Agar; Alanine; Anaerobiosis; Bacteria; Child; Culture Media; Feces; Humans; Infant; Male; Phenylketo

1980
"Physiological phenylketonuria": a biochemical defect caused by delayed maturation of the phenylalanine hydroxylation pathway and by competition with the phenylalanine biosynthetic pathway.
    Medical hypotheses, 1980, Volume: 6, Issue:2

    Topics: Alanine; Bacteria; Drug Resistance, Microbial; Feedback; Humans; Intestinal Mucosa; Models, Theoreti

1980
Comparative analysis of phenylalanine hydroxylase A104D mutant, associated with variant phenylketonuria, and wild-type enzyme.
    Biochemical Society transactions, 1997, Volume: 25, Issue:2

    Topics: Alanine; Aspartic Acid; Chromatography, Affinity; Chromatography, Gel; Humans; Kinetics; Phenylalani

1997
Alanine prevents the decrease of Na+,K+-ATPase activity in experimental phenylketonuria.
    Metabolic brain disease, 1999, Volume: 14, Issue:2

    Topics: Alanine; Animals; Animals, Newborn; Cerebral Cortex; Injections, Subcutaneous; Phenylalanine; Phenyl

1999
Phenylalanine inhibition of the phosphorylation of cytoskeletal proteins from cerebral cortex of young rats is prevented by alanine.
    European journal of clinical investigation, 2000, Volume: 30, Issue:6

    Topics: Age Factors; Alanine; Animals; Cerebral Cortex; Cytoskeletal Proteins; Electrophoresis, Polyacrylami

2000
Platelet Na+, K+-ATPase activity as a possible peripheral marker for the neurotoxic effects of phenylalanine in phenylketonuria.
    Metabolic brain disease, 2000, Volume: 15, Issue:2

    Topics: Alanine; Biomarkers; Blood Platelets; Ca(2+) Mg(2+)-ATPase; Cell Membrane; Humans; Neurotoxins; Phen

2000
Distribution and frequency of pku and hyperphenylalaninemia in eastern and western Austria.
    Irish medical journal, 1976, Sep-30, Volume: 69, Issue:15

    Topics: Alanine; Austria; Humans; Infant, Newborn; Phenylketonurias

1976
The effect of feeding -2-thienylalanine on phenylalanine metabolism in the rhesus monkey.
    Australian and New Zealand journal of medicine, 1973, Volume: 3, Issue:2

    Topics: Alanine; Animals; Animals, Newborn; Body Height; Body Weight; Brain; Cephalometry; Depression, Chemi

1973
Learning in monkeys fed elevated amino acid diets.
    Journal of medical primatology, 1973, Volume: 2, Issue:3

    Topics: Alanine; Amino Acids; Animals; Diet; Discrimination Learning; Female; Food; Glycine; Haplorhini; His

1973
Is the inhibition of glutamic-pyruvic transaminase by phenylalanine one of the causes of hypoglucosemia in phenylketonurics?
    Acta Universitatis Carolinae. Medica. Monographia, 1973, Volume: 56

    Topics: Alanine; Alanine Transaminase; Blood Glucose; Fasting; Gluconeogenesis; Humans; Liver; Liver Extract

1973
[Paper chromatography in the detection of aminoacidopathies].
    Minerva pediatrica, 1971, Dec-22, Volume: 23, Issue:51

    Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Chromatography, Paper; Glycine; Histidin

1971
Amino acid transport and mental retardation.
    Clinical chemistry, 1971, Volume: 17, Issue:11

    Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Animals; Benzyl Compounds; Biological Tr

1971
An investigation of the hyperaminoaciduria in phenylketonuria associated with the feeding of certain commercial low-phenylalanine preparations.
    The British journal of nutrition, 1970, Volume: 24, Issue:2

    Topics: Alanine; Amino Acids; Child; Child, Preschool; Diet Therapy; Female; Humans; Hydro-Lyases; Infant; M

1970