Abstract
Summary: A method was developed to quantitate protein-bound homocyst(e)ine using 2-mercaptoethanol. Protein-bound homocyst(e)ine was discovered in the plasma from normal individuals, ranging from 0.5–2.2 nmole/ml. In two obligatory heterozygotes for classical homocystinuria, plasma protein-bound homocyt(e)ine was 3.5 and 4.8 nmole/ml, respectively. Untreated homozygotes showed approximately a 40-fold increase of plasma protein-bound homocyst(e)ine. Furthermore, using conventional methods, no free homocystine was detectable in the supernatant of plasma precipitate from two classical homocystinuric patients treated with pyridoxine, but plasma protein-bound homocyst(e)ine showed a 10-fold increase. Protein-bound homocyst(e)ine was also demonstrated in the liver, kidney, and brain tissues from a patient with methylenetetrahydrofolate reductase deficiency.
Speculation: The results in this study suggest that determination of proteinbound homocyst(e)ine using 2-mercaptoethanol may provide a more reliable assessment of treatment in patients with homocystinuria and a potentially useful tool for the definition of the carrier state.
Demonstration of protein-bound homocyst(e)ine in various tissues of homocystinuric patients suggests the possibility that this compound may be directly associated with the development of some of the pathologic changes in the tissues
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Kang, SS., Wong, P. & Becker, N. Protein-Bound Homocyst(e)ine in Normal Subjects and in Patients with Homocystinuria. Pediatr Res 13, 1141–1143 (1979). https://doi.org/10.1203/00006450-197910000-00012
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DOI: https://doi.org/10.1203/00006450-197910000-00012
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