Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Antioxidant status in hyperphenylalaninemia

C Sierra1, M A Vilaseca, D Moyano

  • 1Laboratori, Hospital Universitari Sant Joan de Déu, Barcelona, Spain.

Clinica Chimica Acta; International Journal of Clinical Chemistry
|October 6, 1998
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Synergistic inhibition of pneumococcal growth by <i>Dolosigranulum pigrum</i> and <i>Corynebacterium pseudodiphtheriticum:</i> insights into nasopharyngeal microbial interactions.

Microbiology spectrum·2025
Same author

European guidelines on diagnosis and treatment of phenylketonuria: First revision.

Molecular genetics and metabolism·2025
Same author

Nitrate: "the source makes the poison".

Critical reviews in food science and nutrition·2024
Same author

A novel algorithm for optimizing hydrocyclone operations in the decontamination of potentially toxic elements in soils.

Chemosphere·2024
Same author

A novel heuristic tool for selecting the best upgrading conditions for the removal of potentially toxic elements by soil washing.

Journal of hazardous materials·2024
Same author

The Murine Oral Metatranscriptome Reveals Microbial and Host Signatures of Periodontal Disease.

Journal of dental research·2023

Phenylketonuria (PKU) and mild hyperphenylalaninemia patients show lower erythrocyte glutathione peroxidase (GSH-Px) activity, linked to neuropsychological issues in PKU. This antioxidant enzyme deficiency may stem from factors beyond selenium levels.

Area of Science:

  • Biochemistry
  • Metabolic Disorders
  • Neuroscience

Background:

  • Inborn errors of metabolism can cause oxidative stress due to toxic metabolite accumulation and dietary restrictions affecting antioxidant status.
  • Phenylketonuria (PKU) and mild hyperphenylalaninemia are metabolic disorders with potential implications for oxidative stress and antioxidant defense mechanisms.

Purpose of the Study:

  • To investigate erythrocyte antioxidant enzyme activities and tocopherol concentrations in patients with PKU and mild hyperphenylalaninemia.
  • To determine plasma selenium levels in these patient groups and compare them to controls.
  • To explore the relationship between antioxidant status and neuropsychological disorders in these conditions.

Main Methods:

  • Measurement of erythrocyte glutathione peroxidase (GSH-Px) activity and tocopherol concentrations.

Related Experiment Videos

  • Determination of plasma selenium levels.
  • Comparison of biochemical markers and neuropsychological assessments between patient groups (PKU, mild hyperphenylalaninemia) and age-matched controls.

    • Main Results:

    • Significantly lower erythrocyte GSH-Px activity was observed in both PKU and mild hyperphenylalaninemia patients compared to controls (P < 0.001).
    • No significant difference in GSH-Px activity was found between PKU and mild hyperphenylalaninemia groups.
    • Neuropsychological disturbances were more prevalent in PKU patients with lower GSH-Px activity.
    • Plasma selenium levels were normal in both patient groups, suggesting factors other than selenium deficiency contribute to low GSH-Px activity.

    Conclusions:

    • Reduced erythrocyte GSH-Px activity is a characteristic finding in both PKU and mild hyperphenylalaninemia.
    • Low GSH-Px activity is associated with increased neuropsychological disturbances in PKU patients.
    • The observed low GSH-Px activity in these conditions may be influenced by factors such as unbalanced amino acid profiles, rather than solely by selenium status.