s-adenosyl-3-methylthiopropylamine and Mental-Retardation--X-Linked

dcAdoMet (decarboxylated S-adenosylmethionine) is an essential intermediate in the synthesis of polyamines. Its content is normally very low, amounting to less than 5% of that of S-adenosylmethionine itself. It was found that in mice lacking spermine synthase there was a large increase in dcAdoMet and that overexpression of spermine synthase reduced the amount of this nucleoside. There was also an increase in dcAdoMet in cells derived from patients with Snyder-Robinson syndrome, a rare X-linked recessive human disease caused by SMS gene mutations that greatly reduce the content of spermine synthase. These results suggest that there is an inverse relationship between the amount of spermine synthase protein and the content of dcAdoMet and raise the possibility that some of the abnormalities seen in mammals deficient in spermine synthase might be due to changes in dcAdoMet pools.

Topics: Animals; Biogenic Amines; Cells, Cultured; Decarboxylation; Humans; Mental Retardation, X-Linked; Mice; Mice, Knockout; S-Adenosylmethionine; Spermine Synthase