A Biochemical Explanation of Phenyl Acetate Neurotoxicity in Experimental Phenylketonuria

Journal of Neurochemistry

Volumn 45, Issue 5, 1985, Pages 1596-1600

  Loo, Yen Hoong ^a   Potempska, Anna ^a   Wisniewski, Henry M ^a  

^a NEW YORK STATE INSTITUTE FOR BASIC RESEARCH IN DEVELOPMENTAL DISABILITIES   (United States)

Author keywords

Acetyl coenzyme A utilization; Phenyl acetate inhibition; Phenylketonuria

Indexed keywords

ACETYL COENZYME A; PHENYLACETIC ACID; PHENYLALANINE 4 MONOOXYGENASE;

3 HYDROXYBUTYRIC ACID C 14; ANIMAL CELL; ANIMAL MODEL; CENTRAL NERVOUS SYSTEM; INTOXICATION; NERVOUS SYSTEM; NEUROTOXICITY; NONHUMAN; PHENYLKETONURIA; PRIORITY JOURNAL; RAT;

3-HYDROXYBUTYRIC ACID; ACETYL COENZYME A; ANIMAL; BRAIN; DISEASE MODELS, ANIMAL; FEMALE; GLYCOPROTEINS; HYDROXYBUTYRATES; KINETICS; MALE; PHENYLACETATES; PHENYLKETONURIAS; RATS; RATS, INBRED STRAINS; SIALIC ACIDS; SUPPORT, U.S. GOV'T, P.H.S.;

EID: 0022227579     PISSN: 00223042     EISSN: 14714159     Source Type: Journal    
DOI: 10.1111/j.1471-4159.1985.tb07232.x     Document Type: Article

References (39)

1. Morphologic and histoanatomic observations of the brain in untreated phenylketonuria
2. 14C] into rat brain gangliosides
3. D‐3‐Hydroxybutyrate: a major precursor of amino acids in developing rat brain
4. Ketone‐bodies are precursors of sterols and fatty acids in the developing rat
5. A simple method for the isolation and purification of total lipids from animal tissues
6. Phenylacetate and the enduring behavioral deficit in experimental phenylketonuria
7. Ketone‐body utilization by adult and suckling rat brain in vivo
8. Chemical characterization of a neural cell adhesion molecule purified from embryonic brain membranes
9. Acetoacetate metabolism in infant and adult rat brain in vitro
10. The course of ketosis and the activity of key enzymes of ketogenesis and ketone‐body utilization during development of the postnatal rat
11. Gas chromatographic determination of aromatic acid metabolites of phenylalanine in brain
12. Myelin deficiency in experimental phenylketonuria: contribution of the aromatic acid metabolites of phenylalanine
13. Vulnerability of the immature rat brain to phenylacetate intoxication: tissue permeability to phenylacetate
14. Vulnerability of the immature rat brain to phenylacetate intoxication: postnatal development of the detoxication mechanism
15. Phenylacetate and brain dysfunction in experimental phenylketonuria: synaptic development
16. Identification of metabolites of phenylacetic acid in rat brain by plasma desorption mass spectrometry
17. Experimental maternal phenylketonuria: an examination of two animal models
18. Use of butylacetate in determination of sialic acid
19. A quantitative ultrastructural study of the effects of phenylacetate on synaptic organization in the developing rat cerebral cortex
20. Activities of enzymes of ketone‐body utilization in brain and other tissues of suckling rats
21. Effect of hyperphenylalaninemia on lipid synthesis from ketone‐bodies by rat brain
22. Lipogenesis from ketonebodies in rat brain. Evidence for conversion of acetoacetate into acetyl‐CoA in the cytosol
23. On the possible mechanism of phenylacetate neurotoxicity: inhibition of choline acetyltransferease by phenylacetyl‐CoA
24. Synthesis of glycoproteins and gangliosides in developing rat brain
25. Purkinje cell dendritic development in experimental phenylketonuria. A quantitative analysis
26. Experimental phenylketonuria: effect of phenylacetate intoxication on number of synapses in the cerebellar cortex of the rat
27. The pattern of mammalian gangliosides. II. Evaluation of the extraction procedures, postmortem changes and the effect of formalin preservation
28. Formation and turnover of major brain gangliosides during development
29. Quantitative estimation of sialic acids. II. A colorimetric resorcinol‐HCl method
30. 14C]hydroxybutyrate by malnourished rat pups
31. Metabolism of D‐ and L‐3‐Hydroxybutyrate in developing rat brain
32. Utilization of L‐(+)‐3‐hydroxybutyrate, D‐(–)‐3‐hydroxybutyrate, acetoacetate and glucose for respiration and lipid synthesis in the 18‐day‐old rat
33. The in vivo utilization of acetoacetate, D‐(–)‐3‐hydroxybutyrate and glucose for lipid synthesis in brain in the 18‐day‐old rat
34. Neuropathology of phenylacetate poisoning in rats: an experimental model of phenylketonuria
35. 18 cartridges during the isolation of gangliosides
36. Activities of enzymes involved in acetoacetate utilization in adult mammalian tissues
37. Excretion of conjugated phenylacetic in phenylketonuria
38. Ketone‐bodies serve as important precursors of brain lipids in the developing rat