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hydrogen carbonate and Phenylketonurias

hydrogen carbonate has been researched along with Phenylketonurias in 4 studies

Bicarbonates: Inorganic salts that contain the -HCO3 radical. They are an important factor in determining the pH of the blood and the concentration of bicarbonate ions is regulated by the kidney. Levels in the blood are an index of the alkali reserve or buffering capacity.
hydrogencarbonate : The carbon oxoanion resulting from the removal of a proton from carbonic acid.

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

ExcerptRelevanceReference
"Low-dose continuous infusions of [2H5]phenylalanine, [1-13C]propionate, and [1-13C]leucine were used to quantitate phenylalanine hydroxylation in phenylketonuria (PKU, four subjects), propionate oxidation in methylmalonic acidaemia (MMA, four subjects), and propionic acidaemia (PA, four subjects) and leucine oxidation in maple syrup urine disease (MSUD, four subjects)."3.68In vivo enzyme activity in inborn errors of metabolism. ( Halliday, D; Leonard, JV; Thompson, GN; Walter, JH, 1990)

Research

Studies (4)

TimeframeStudies, this research(%)All Research%
pre-19903 (75.00)18.7374
1990's1 (25.00)18.2507
2000's0 (0.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
PASIEKA, AE1
THOMAS, ME1
LOGAN, JE1
ALLEN, RH1
Thompson, GN1
Walter, JH1
Leonard, JV1
Halliday, D1
Patel, MS2
Grover, WD1
Auerbach, VH1

Other Studies

4 other studies available for hydrogen carbonate and Phenylketonurias

ArticleYear
SPECIFIC DETECTION OF PHENYLALANINE FOLLOWING CHROMATOGRAPHIC SEPARATION FROM SERUM AND URINE OF PHENYLKETONURIC.
    Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine (New York, N.Y.), 1964, Volume: 115

    Topics: Amino Acid Metabolism, Inborn Errors; Bicarbonates; Blood Chemical Analysis; Chromatography; Humans;

1964
In vivo enzyme activity in inborn errors of metabolism.
    Metabolism: clinical and experimental, 1990, Volume: 39, Issue:8

    Topics: Adult; Bicarbonates; Carbon Isotopes; Child; Child, Preschool; Deuterium; Female; Humans; Hydroxylat

1990
The effect of phenylpyruvate on pyruvate metabolism in rat brain.
    The Biochemical journal, 1972, Volume: 128, Issue:3

    Topics: Animals; Bicarbonates; Brain; Carbon Isotopes; Culture Media; Freeze Drying; Humans; In Vitro Techni

1972
Pyruvate metabolism by homogenates of human brain: effects of phenylpyruvate and implications for the etiology of the mental retardation in phenylketonuria.
    Journal of neurochemistry, 1973, Volume: 20, Issue:2

    Topics: Bicarbonates; Brain; Carbon Isotopes; Decarboxylation; Humans; In Vitro Techniques; Intellectual Dis

1973