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

Galactose utilization in galactosemia.

J C Petricciani, M K Binder, C R Merril

    Science (New York, N.Y.)
    |March 24, 1972
    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

    Dental amalgam fillings and mercury vapor safety limits in American adults.

    Human & experimental toxicology·2022
    Same author

    Mitochondrial dysfunction, impaired oxidative-reduction activity, degeneration, and death in human neuronal and fetal cells induced by low-level exposure to thimerosal and other metal compounds.

    Toxicological and environmental chemistry·2014
    Same author

    A significant dose-dependent relationship between mercury exposure from dental amalgams and kidney integrity biomarkers: a further assessment of the Casa Pia children's dental amalgam trial.

    Human & experimental toxicology·2012
    Same author

    The acceptability of continuous cell lines: A personal & historical perspective.

    Cytotechnology·2012
    Same author

    A comprehensive review of mercury provoked autism.

    The Indian journal of medical research·2008
    Same author

    An overview of viral and viral-like agents in cell culture systems.

    Cytotechnology·2008
    Same journal

    Erratum for the Research Article "Detecting supramolecular organic nanoparticles during heat wave".

    Science (New York, N.Y.)·2026
    Same journal

    Local signals, systemic decline.

    Science (New York, N.Y.)·2026
    Same journal

    The mechanics of liver regeneration.

    Science (New York, N.Y.)·2026
    Same journal

    Computing in a memory with physics.

    Science (New York, N.Y.)·2026
    Same journal

    Retraction.

    Science (New York, N.Y.)·2026
    Same journal

    Making time.

    Science (New York, N.Y.)·2026
    See all related articles

    Human galactosemic fibroblasts can process galactose, but slower than normal cells. High glucose levels significantly inhibit galactose utilization in both cell types.

    Area of Science:

    • Biochemistry
    • Cell Biology
    • Metabolic Disorders

    Background:

    • Galactosemia is a genetic disorder affecting galactose metabolism.
    • The enzyme galactose-1-phosphate uridyltransferase (GALT) is crucial for galactose utilization.
    • Deficiency in GALT activity leads to galactose accumulation and toxicity.

    Purpose of the Study:

    • To investigate galactose metabolism in human galactosemic fibroblasts lacking GALT activity.
    • To assess the impact of glucose on galactose utilization in both normal and galactosemic cells.

    Main Methods:

    • Culturing human fibroblasts with and without galactosemia.
    • Utilizing [1-(14)C]galactose to trace metabolic conversion.
    • Measuring the production of (14)CO(2) as an indicator of galactose metabolism.

    Related Experiment Videos

  • Assessing the effect of physiological glucose concentrations on galactose utilization.

    • Main Results:

    • Galactosemic fibroblasts converted [1-(14)C]galactose to (14)CO(2) at a rate comparable to normal cells, albeit significantly slower.
    • Galactose utilization in both normal and galactosemic fibroblasts was markedly inhibited by glucose at physiological concentrations.

    Conclusions:

    • Human galactosemic fibroblasts possess alternative pathways for galactose metabolism, independent of GALT.
    • Glucose plays a significant regulatory role in galactose utilization, impacting both normal and galactosemic metabolic pathways.