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Endothelial cell dysfunction in homocystinuria.

P G de Groot, C Willems, G H Boers

    European Journal of Clinical Investigation
    |October 1, 1983
    PubMed
    Summary
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    Vascular endothelial cells from homocystinuria heterozygotes show reduced function and viability when exposed to sulfur-containing amino acids like methionine and homocystine.

    Area of Science:

    • Biochemistry
    • Cell Biology
    • Vascular Biology

    Background:

    • Homocystinuria is a genetic disorder affecting sulfur-amino acid metabolism.
    • Endothelial cells play a crucial role in vascular health and are exposed to circulating metabolites.

    Observation:

    • Venous endothelial cells were isolated from an obligate heterozygote for homocystinuria and cultured.
    • These cells were exposed to methionine and homocystine and compared to normal endothelial cells.
    • Cell viability (51Cr release) and platelet adherence were measured.

    Findings:

    • Heterozygote endothelial cells exhibited significantly increased 51Cr release and platelet adherence when exposed to methionine or homocystine compared to normal cells.
    • Normal endothelial cells showed sensitivity to homocysteine.

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  • The study suggests a partial deficiency in cystathionine synthase in endothelial cells from the heterozygote.

    • Implications:

    • Endothelial cells from homocystinuria heterozygotes are more susceptible to injury from toxic sulfur-containing amino acids.
    • This cellular dysfunction may contribute to vascular complications in homocystinuria.
    • Understanding endothelial cell vulnerability is key to developing targeted therapies for homocystinuria.