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Plasma cyst(e)ine in homocyst(e)inemia

M H Malloy, D K Rassin, G E Gaull

    The American Journal of Clinical Nutrition
    |December 1, 1981
    PubMed
    Summary

    Plasma homocysteine in patients with homocystinemia displaces cysteine from protein binding sites. Most cysteine exists as a mixed disulfide with homocysteine, not free or protein-bound.

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    Area of Science:

    • Biochemistry
    • Metabolic Disorders
    • Clinical Chemistry

    Background:

    • Plasma cyst(e)ine exists in free and protein-bound forms.
    • Metabolic states can alter the distribution of plasma cyst(e)ine.
    • Homocystinemia is a metabolic disorder characterized by elevated homocysteine levels.

    Purpose of the Study:

    • To investigate the impact of homocystinemia on plasma cyst(e)ine distribution.
    • To quantify the forms of cyst(e)ine present in the plasma of homocystinemia patients.

    Main Methods:

    • Development of a method to measure plasma protein-bound 1/2-cystine.
    • Analysis of plasma samples from five patients with homocystinemia.

    Main Results:

    • In homocystinemia patients, 1/2-homocystine replaced cysteine on plasma protein sulfhydryl-binding sites.
    • The majority of plasma cyst(e)ine (5.4 +/- 0.9 mumol/dl) was found as a mixed disulfide with 1/2-homocystine.
    • Free plasma cyst(e)ine levels were significantly reduced in these patients.

    Conclusions:

    • Homocystinemia profoundly alters plasma cyst(e)ine metabolism and distribution.
    • The formation of mixed disulfides with homocysteine is a key feature of cyst(e)ine metabolism in homocystinemia.
    • This altered distribution may have implications for cysteine availability and related metabolic pathways.

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