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Protein-Bound Homocyst(e)ine in Normal Subjects and in Patients with Homocystinuria
姜洙祥 계명대출판부 1979 童山申泰植博士古稀紀念論叢 Vol.S No.-
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 to 2.2nmol/ml. In two obligatory heterozygotes for classical homocystinuria, plasma protein-bound homocyst(e)ine was 3.5 and 4.8nmol/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 cIassical 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.