Page last updated: 2024-10-17

lactic acid and BH4 Deficiency

lactic acid has been researched along with BH4 Deficiency in 6 studies

Lactic Acid: A normal intermediate in the fermentation (oxidation, metabolism) of sugar. The concentrated form is used internally to prevent gastrointestinal fermentation. (From Stedman, 26th ed)
2-hydroxypropanoic acid : A 2-hydroxy monocarboxylic acid that is propanoic acid in which one of the alpha-hydrogens is replaced by a hydroxy group.

Research Excerpts

ExcerptRelevanceReference
"It has been suggested that the hyperphenylalaninaemia in patients with PKU reduces complex I (NADH:ubiquinone reductase) activity of the mitochondrial respiratory chain (MRC) and/or biosynthesis of coenzyme Q(10) (CoQ(10)), which acts as an electron carrier in the MRC, leading to impaired energy metabolism in the brain of patients with PKU and hence the neurological pathology."1.35Assessment of mitochondrial respiratory chain function in hyperphenylalaninaemia. ( Hargreaves, I; Kyprianou, N; Lee, P; Murphy, E, 2009)

Research

Studies (6)

TimeframeStudies, this research(%)All Research%
pre-19902 (33.33)18.7374
1990's1 (16.67)18.2507
2000's2 (33.33)29.6817
2010's1 (16.67)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Kyprianou, N1
Murphy, E1
Lee, P1
Hargreaves, I1
Zhang, D1
Li, W1
Zhang, J1
Tang, W1
Qian, C1
Feng, M1
Chu, Q1
Ye, J1
PRESCOTT, BA1
BOREK, E1
Bal, D1
Kraska-Dziadecka, A1
Gradowska, W1
Gryff-Keller, A1
Mönch, E1
Herrmann, ME1
Brösicke, H1
Schöffer, A1
Keller, M1
Kolodny, EH1
Yatziv, S1

Other Studies

6 other studies available for lactic acid and BH4 Deficiency

ArticleYear
Assessment of mitochondrial respiratory chain function in hyperphenylalaninaemia.
    Journal of inherited metabolic disease, 2009, Volume: 32, Issue:2

    Topics: Adult; Amino Acid Metabolism, Inborn Errors; Cell Line, Tumor; Cells, Cultured; Culture Media; Elect

2009
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
Studies on oligophrenia phenylpyruvica; microbiological determination of L- and D-phenylalanine and of phenyl lactic acid.
    The Journal of biological chemistry, 1949, Volume: 181, Issue:1

    Topics: Acids; Body Fluids; Intellectual Disability; Lactic Acid; Phenylalanine; Phenylketonurias; Urine

1949
Investigation of a wide spectrum of inherited metabolic disorders by 13C NMR spectroscopy.
    Acta biochimica Polonica, 2008, Volume: 55, Issue:1

    Topics: Biomarkers; Canavan Disease; Glutarates; Hemiterpenes; Humans; Lactic Acid; Magnetic Resonance Spect

2008
Utilisation of amino acid mixtures in adolescents with phenylketonuria.
    European journal of pediatrics, 1996, Volume: 155 Suppl 1

    Topics: Adolescent; Adult; Amino Acids; Arginine; Blood Glucose; Diet, Protein-Restricted; Dietary Proteins;

1996
Laboratory approaches for inherited neurometabolic diseases.
    Developmental medicine and child neurology, 1985, Volume: 27, Issue:2

    Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Galactosemias; Homocystinuria; Humans; Lactates; Lact

1985