Abstract
Extract: Preincubation of fibroblasts with isoleucine before freeze-thawing inhibited the interconversion of glycine and serine. Inhibition was not seen when isoleucine was added after the cells were broken, which suggests that isoleucine must be metabolized to form an inhibitor substance. Tiglic acid was shown to accumulate during this preincubation period and was demonstrated to inhibit interconversion. Methylene tetrahydrofolate production was also reduced by preincubation with isoleucine or incubation with tiglic acid but this decreased production could not be proven to be related to inhibition of serine-glycine interconversion.
Speculation: The metabolic consequences of the three enzyme deficiencies associated with the “ketotic hyperglycinemia” syndrome are protean. The data presented in this paper suggest that some of the problems may be related to secondary inhibition of tetrahydrofolate metabolism. Folate therapy may be of some value in the clinical management of patients with this syndrome.
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Hillman, R., Otto, E. Inhibition of Glycine-Serine Interconversion in Cultured Human Fibroblasts by Products of Isoleucine Catabolism. Pediatr Res 8, 941–945 (1974). https://doi.org/10.1203/00006450-197412000-00005
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DOI: https://doi.org/10.1203/00006450-197412000-00005
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