Abstract: . . .  the cysteinyl groups of albumin and other plasma proteins, and only a small fraction (about 10%) of homocysteine in plasma is in the free sulfhydryl form. The free mixed homocysteine cysteine disulfide and the disulfide of homocysteine, homocystine, are not detect- able in normal plasma, but are present in plasma and urine of patients with homocystinuria. Whether free homocysteine could interact with cysteinyl groups of abnormal fibrillin-1 molecules . . .
. . .  currently in progress worldwide to determine whether this approach will prevent mortality and morbidity in atherosclerosis. Positive results from these trials may suggest a similar approach to preventing morbidity and mortality in Marfan Syndrome, opening a further field of investigation. References 1. Mudd SH, Skovby F, Levy HL et al. The natural history of homo- cystinuria due to cystathionine beta synthase deficiency. Am J Hum Genet . . .
. . .  progress worldwide to determine whether this approach will prevent mortality and morbidity in atherosclerosis. Positive results from these trials may suggest a similar approach to preventing morbidity and mortality in Marfan Syndrome, opening a further field of investigation. References 1. Mudd SH, Skovby F, Levy HL et al. The natural history of homo- cystinuria due to cystathionine beta synthase deficiency. Am J Hum Genet 1985; 37 . . .
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. . .  2. Schimke RN, McKusick VA, Huang T et al. Homocystinuria. Studies of 20 families with 38 affected members. JAMA 1965; 193 :7119. 3. McCully KS. Vascular pathology of homocysteinemia: implications for the pathogenesis of arteriosclerosis . Am J Pathol 1969; 56 :11128. 4. Gibson JB, Carson NAJ, Neill DW. Pathological findings in homocystinuria. J Clin Pathol 1964; 17 :42737. 5. McCully KS. Homocysteine and vascular disease. Nature Med 1996; . . .
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