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Incomplete understanding of brain biochemistry represents a major knowledge gap to develop new treatments. We examined the biochemistry in blood, liver, cortex, hippocampus, and cerebellum of a mouse model homozygous for the\n                    <jats:italic>Gldc<\/jats:italic>\n                    variant p.Ala394Val. Glycine was increased in all compartments and caused increased brain neurotoxic metabolites guanidinoacetate and methylglyoxal, and also N\u2010acetylglycine and cystathionine. The glycine extruding transporter Slc6a20 was increased. There was reduced one\u2010carbon folate charging with secondarily reduced methionine in the cortex, and reduced alternative one\u2010carbon donors L\u2010serine and formate. Serine deficiency was associated with reduced amounts of sphingosine, sphingomyelin, and ceramide species important for myelination, but not phosphatidylserines. There was a region\u2010specific deficiency of D\u2010serine in the cortex and hippocampus. This difference, also present in humans, was strain\u2010 and age\u2010related, most evident in young J129X1\/SvJ mice, reflecting symptomatology. There was no evidence of oxidative stress or a bioenergetic defect. The biochemistry of the nonketotic hyperglycinemia mouse model can be traced to three components: increased glycine, reduced folate one\u2010carbon charging, and decreased L\u2010 and D\u2010serine. These changes will need to be addressed in new therapeutic approaches.\n                  <\/jats:p>","DOI":"10.1002\/jimd.70137","type":"journal-article","created":{"date-parts":[[2026,1,28]],"date-time":"2026-01-28T10:15:13Z","timestamp":1769595313000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["A Nonketotic Hyperglycinemia Mouse Shows Wide\u2010Ranging Biochemical Consequences of Elevated Glycine, Reduced Folate One\u2010Carbon Charging, and Serine Deficiency"],"prefix":"10.1002","volume":"49","author":[{"given":"Michael A.","family":"Swanson","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Section of Clinical Genetics and Metabolism University of Colorado Anschutz Medical Campus  Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hua","family":"Jiang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Section of Clinical Genetics 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