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• Journal

  The Open nanomedicine journal

  Digital Access Bentham Open v. 1-3, 2008-10.
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• Book

  The evolution of poverty in Canada, 1970-1985

  by Pierre Perron and François Vaillancourt.

  Print [1988]
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• Article

  Homocystinuria: pathogenetic mechanisms.

  Grieco AJ.

  Am J Med Sci. 1977 Mar-Apr;273(2):120-32.

  Homocystinuria with elevated plasma homocysteine and methionine levels is the result of deficient activity of cystathionine synthetase, the enzyme catalyzing conversion of homocysteine to cystathionine. It is inherited as an autosomal recessive trait with a worldwide distribution. The major clinical manifestations result from the elevated plasma homocysteine level. The excitotoxic effect of homocysteic acid accounts for mental retardation and seizures. Interference with collagen cross-linking by sulfhydryl groups of homocysteine causes ectopia lentis and skeletal deformities. Sulfation factor-like effects contribute to disruption of vascular endothelium, which is followed by platelet thrombosis and widespread arterial and venous occlusions. Low methionine homocystinuria, with deficient remethylation of homocysteine, results from deranged vitamin B(12) metabolism and from deficient 5,10-methylene-tetrahydrofolate reductase. Administration of azaribine produces homocystinuria by mechanism not yet elucidated.

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